Mitochondrial fatty acid beta-oxidation (Homo sapiens)

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12, 40, 50, 5124, 29362536302, 1721362529, 542542, 521038, 41, 5324, 29361024, 29251, 11, 16, 23, 28...258, 134, 6, 15, 20, 22...31, 34, 35, 39, 47...29, 54252524, 2910252510181814, 2719, 36103, 5, 7, 9, 432529, 5433, 44cytosolmitochondrial matrixendoplasmic reticulum lumenMUT PCCB DCI dimerMCFA, LCFAHADHA ECHS1 hexamerECHS1 TrifunctionalProteinMCFA-CoAH+S-2MDPDA-CoAFADAc-CoAMECR MCFA HADH 3HB-CoAAc-CoACoA-SHACADM tetramerECHS1 hexamerHADHB CoA-SHACSF2TrifunctionalProteinLAU-CoANAD+ACADVL dimerCrotonoyl-CoAMYS-CoAACADS tetramer2-trans-Dodecenoyl-CoAFADH2H+trans,cis-Lauro-2,6-dienoyl-CoAH+HADHA FADH2FADH2ATPMCFA-CoA LCFA-CoA Ac-CoAACSM3,ACSM6FADH2DBI, (ACBD7)MCFA-CoA NAD+MCFA ACAD11HADHB ACAD10CoA-SHH+NADHcis,cis-3,6-Dodecadienoyl-CoACoA-SH3-trans-decenoyl-CoAMal-PPANT-S112-NDUFAB1NADHNADHMCFA-CoA, LCFA-CoAACAA2 tetramertrans-Hexadec-2-enoyl-CoANADH(S)-Hydroxydecanoyl-CoAACADS tetramerHADH dimerMCATACADM tetramerBH-CoATrifunctionalProtein3OA-CoAOctanoyl-CoAFADFADH2AdoCbl (S)-Hydroxyoctanoyl-CoADBI,ACBD7:MCFA-CoA,LCFA-CoAACBD6:LCFA-CoAadenosine5'-monophosphateH2OAc-CoACoA-SHACADL DECR1 MECR dimerNADHPPANT-S112-NDUFAB1FAD3-Oxohexanoyl-CoAHADHB LCFA-CoA FADH+2xMMAA:2xMUT:AdoCblDBI TrifunctionalProteinH2OADPCoA-SHH+ACOT11 NAD+Btn-PCCA MCFA-CoA ACAA2 Cytosolic ACOTs,THEM4ECHS1 hexamerACOT1 ACBD6 MCFA-CoA, LCFA-CoAMCFA, LCFAACADM NAD+FADACADS PPiACSM3 ECI1 Ac-CoACoA-SHDEC-CoAH+H2OACBD6TrifunctionalProteinH2OHADHA 4-cis-decenoyl-CoAACOT7 LCFA-CoA HADHB DBI ACA-CoAFADH2FADH2FADH2ACOT2 H+NAD+THEM4 FADATPH2OACBD7 NAD+ACOT2,9, THEM4,5dimerH2OFADH2ACSM6 ACADL tetramerAc-CoAadenosine5'-monophosphateECHS1 PROP-CoAACOT9 NAD+NADHACBD7 6x(Btn-PCCA:PCCB)ECHS1 hexamerPalmCoA3-Oxotetradecanoyl-CoA3-Oxodecanoyl-CoAHADH dimerNADPHACOT13 HADHA CoA-SHFADH2Ac-CoAacyl-CoA2-trans-4-cis-decadienoyl-CoACoA-SHH+H+H+FADECHS1 ECHS1 (S)-3-Hydroxytetradecanoyl-CoAHADH dimerHADHB MEMA-CoA(S)-Hydroxyhexanoyl-CoAPCTP(S)-3-Hydroxyhexadecanoyl-CoA3-Oxododecanoyl-CoANAD+DECR1 tetramerLCFA trans-Oct-2-enoyl-CoAPPiH2ONADH3-Oxooctanoyl-CoANADHTrifunctionalProteinATPS-2MPDA-CoAFADMCFABT-CoAACADM CoA-SHNAD+ACADS FADPiFADH2LCFA MCFA-CoA ACADL FADH2LCFA-CoA NADP+HADHB Malonyl-CoAHADH FADNADHH2OHADHA FADTHEM5 H2OAc-CoAFADLIN-CoANADHACADVL ACADL ACADL tetramerECHS1 hexamerL-MM-CoAMCEEHX-CoADHB-CoAtdec2-CoASUCC-CoAtrans-Tetradec-2-enoyl-CoACoA-SHBUTtrans-Hex-2-enoyl-CoAMMAA MCFA-CoA, LCFA-CoALCFA-CoALCFA-CoA HADH H2OH2OACOT7L ACOT12 CO2ACADL tetramerHADHA 3-Oxopalmitoyl-CoAECHS1 (S)-3-Hydroxydodecanoyl-CoANAD+8, 13531919533819


Description

Beta-oxidation begins once fatty acids have been imported into the mitochondrial matrix by carnitine acyltransferases. The beta-oxidation spiral of fatty acids metabolism involves the repetitive removal of two carbon units from the fatty acyl chain. There are four steps to this process: oxidation, hydration, a second oxidation, and finally thiolysis. The last step releases the two-carbon acetyl-CoA and a ready primed acyl-CoA that takes another turn down the spiral. In total each turn of the beta-oxidation spiral produces one NADH, one FADH2, and one acetyl-CoA.

Further oxidation of acetyl-CoA via the tricarboxylic acid cycle generates additional FADH2 and NADH. All reduced cofactors are used by the mitochondrial electron transport chain to form ATP. The complete oxidation of a fatty acid molecule produces numerous ATP molecules. Palmitate, used as the model here, produces 129 ATPs.<p>Beta-oxidation pathways differ for saturated and unsaturated fatty acids. The beta-oxidation of saturated fatty acids requires four different enzymatic steps. Beta-oxidation produces and consumes intermediates with a trans configuration; unsaturated fatty acids that have bonds in the cis configuration require three separate enzymatic steps to prepare these molecules for the beta-oxidation pathway. View original pathway at Reactome.</div>

Comments

Reactome-Converter 
Pathway is converted from Reactome ID: 77289
Reactome-version 
Reactome version: 75
Reactome Author 
Reactome Author: Gillespie, Marc E

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Bibliography

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History

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CompareRevisionActionTimeUserComment
117793view14:08, 22 May 2021EweitzModified title
114722view16:20, 25 January 2021ReactomeTeamReactome version 75
113166view11:23, 2 November 2020ReactomeTeamReactome version 74
112394view15:33, 9 October 2020ReactomeTeamReactome version 73
101298view11:18, 1 November 2018ReactomeTeamreactome version 66
100835view20:49, 31 October 2018ReactomeTeamreactome version 65
100376view19:24, 31 October 2018ReactomeTeamreactome version 64
99923view16:08, 31 October 2018ReactomeTeamreactome version 63
99478view14:40, 31 October 2018ReactomeTeamreactome version 62 (2nd attempt)
99132view12:40, 31 October 2018ReactomeTeamreactome version 62
93259view11:18, 9 August 2017ReactomeTeamNew pathway

External references

DataNodes

View all...
NameTypeDatabase referenceComment
(S)-3-Hydroxydodecanoyl-CoAMetaboliteCHEBI:27668 (ChEBI)
(S)-3-Hydroxyhexadecanoyl-CoAMetaboliteCHEBI:27402 (ChEBI)
(S)-3-Hydroxytetradecanoyl-CoAMetaboliteCHEBI:27466 (ChEBI)
(S)-Hydroxydecanoyl-CoAMetaboliteCHEBI:28325 (ChEBI)
(S)-Hydroxyhexanoyl-CoAMetaboliteCHEBI:28276 (ChEBI)
(S)-Hydroxyoctanoyl-CoAMetaboliteCHEBI:28632 (ChEBI)
2-trans-4-cis-decadienoyl-CoAMetaboliteCHEBI:29119 (ChEBI)
2-trans-Dodecenoyl-CoAMetaboliteCHEBI:15471 (ChEBI)
2xMMAA:2xMUT:AdoCblComplexR-HSA-3159272 (Reactome)
3-Oxodecanoyl-CoAMetaboliteCHEBI:28528 (ChEBI)
3-Oxododecanoyl-CoAMetaboliteCHEBI:27868 (ChEBI)
3-Oxohexanoyl-CoAMetaboliteCHEBI:27648 (ChEBI)
3-Oxooctanoyl-CoAMetaboliteCHEBI:28264 (ChEBI)
3-Oxopalmitoyl-CoAMetaboliteCHEBI:15491 (ChEBI)
3-Oxotetradecanoyl-CoAMetaboliteCHEBI:28726 (ChEBI)
3-trans-decenoyl-CoAMetaboliteCHEBI:29126 (ChEBI)
3HB-CoAMetaboliteCHEBI:15453 (ChEBI)
3OA-CoAMetaboliteCHEBI:15489 (ChEBI)
4-cis-decenoyl-CoAMetaboliteCHEBI:29140 (ChEBI)
6x(Btn-PCCA:PCCB)ComplexR-HSA-71026 (Reactome)
ACA-CoAMetaboliteCHEBI:15345 (ChEBI)
ACAA2 ProteinP42765 (Uniprot-TrEMBL)
ACAA2 tetramerComplexR-HSA-8874746 (Reactome)
ACAD10ProteinQ6JQN1 (Uniprot-TrEMBL)
ACAD11ProteinQ709F0 (Uniprot-TrEMBL)
ACADL ProteinP28330 (Uniprot-TrEMBL)
ACADL tetramerComplexR-HSA-77258 (Reactome)
ACADM ProteinP11310 (Uniprot-TrEMBL)
ACADM tetramerComplexR-HSA-77335 (Reactome)
ACADS ProteinP16219 (Uniprot-TrEMBL)
ACADS tetramerComplexR-HSA-77316 (Reactome)
ACADVL ProteinP49748 (Uniprot-TrEMBL)
ACADVL dimerComplexR-HSA-77279 (Reactome)
ACBD6 ProteinQ9BR61 (Uniprot-TrEMBL)
ACBD6:LCFA-CoAComplexR-HSA-8848271 (Reactome)
ACBD6ProteinQ9BR61 (Uniprot-TrEMBL)
ACBD7 ProteinQ8N6N7 (Uniprot-TrEMBL)
ACOT1 ProteinQ86TX2 (Uniprot-TrEMBL)
ACOT11 ProteinQ8WXI4 (Uniprot-TrEMBL)
ACOT12 ProteinQ8WYK0 (Uniprot-TrEMBL)
ACOT13 ProteinQ9NPJ3 (Uniprot-TrEMBL)
ACOT2 ProteinP49753 (Uniprot-TrEMBL)
ACOT2,9, THEM4,5 dimerComplexR-HSA-5690051 (Reactome)
ACOT7 ProteinO00154 (Uniprot-TrEMBL)
ACOT7L ProteinQ6ZUV0 (Uniprot-TrEMBL)
ACOT9 ProteinQ9Y305 (Uniprot-TrEMBL)
ACSF2ProteinQ96CM8 (Uniprot-TrEMBL)
ACSM3 ProteinQ53FZ2 (Uniprot-TrEMBL)
ACSM3,ACSM6ComplexR-HSA-8875066 (Reactome)
ACSM6 ProteinQ6P461 (Uniprot-TrEMBL)
ADPMetaboliteCHEBI:456216 (ChEBI)
ATPMetaboliteCHEBI:30616 (ChEBI)
Ac-CoAMetaboliteCHEBI:15351 (ChEBI)
AdoCbl MetaboliteCHEBI:18408 (ChEBI)
BH-CoAMetaboliteCHEBI:65088 (ChEBI)
BT-CoAMetaboliteCHEBI:15517 (ChEBI)
BUTMetaboliteCHEBI:30772 (ChEBI)
Btn-PCCA ProteinP05165 (Uniprot-TrEMBL)
CO2MetaboliteCHEBI:16526 (ChEBI)
CoA-SHMetaboliteCHEBI:15346 (ChEBI)
Crotonoyl-CoAMetaboliteCHEBI:15473 (ChEBI)
Cytosolic ACOTs, THEM4ComplexR-HSA-5690052 (Reactome)
DBI ProteinP07108 (Uniprot-TrEMBL)
DBI, (ACBD7)ComplexR-HSA-8848239 (Reactome)
DBI,ACBD7:MCFA-CoA,LCFA-CoAComplexR-HSA-8848291 (Reactome)
DCI dimerComplexR-HSA-110060 (Reactome)
DEC-CoAMetaboliteCHEBI:28493 (ChEBI)
DECR1 ProteinQ16698 (Uniprot-TrEMBL)
DECR1 tetramerComplexR-HSA-110061 (Reactome)
DHB-CoAMetaboliteCHEBI:85880 (ChEBI)
ECHS1 ProteinP30084 (Uniprot-TrEMBL)
ECHS1 hexamerComplexR-HSA-71048 (Reactome)
ECI1 ProteinP42126 (Uniprot-TrEMBL)
FADMetaboliteCHEBI:16238 (ChEBI)
FADH2MetaboliteCHEBI:17877 (ChEBI)
H+MetaboliteCHEBI:15378 (ChEBI)
H2OMetaboliteCHEBI:15377 (ChEBI)
HADH ProteinQ16836 (Uniprot-TrEMBL)
HADH dimerComplexR-HSA-71052 (Reactome)
HADHA ProteinP40939 (Uniprot-TrEMBL)
HADHB ProteinP55084 (Uniprot-TrEMBL)
HX-CoAMetaboliteCHEBI:27540 (ChEBI)
L-MM-CoAMetaboliteCHEBI:15465 (ChEBI)
LAU-CoAMetaboliteCHEBI:15521 (ChEBI)
LCFA MetaboliteCHEBI:15904 (ChEBI)
LCFA-CoA MetaboliteCHEBI:33184 (ChEBI)
LCFA-CoAMetaboliteCHEBI:33184 (ChEBI)
LIN-CoAMetaboliteCHEBI:15530 (ChEBI)
MCATProteinQ8IVS2 (Uniprot-TrEMBL)
MCEEProteinQ96PE7 (Uniprot-TrEMBL)
MCFA MetaboliteCHEBI:59554 (ChEBI)
MCFA, LCFAComplexR-ALL-5690064 (Reactome)
MCFA, LCFAComplexR-ALL-5690519 (Reactome)
MCFA-CoA MetaboliteCHEBI:61907 (ChEBI)
MCFA-CoA, LCFA-CoAComplexR-ALL-5690044 (Reactome)
MCFA-CoA, LCFA-CoAComplexR-ALL-5690540 (Reactome)
MCFA-CoA, LCFA-CoAComplexR-ALL-8848288 (Reactome)
MCFA-CoAMetaboliteCHEBI:61907 (ChEBI)
MCFAMetaboliteCHEBI:59554 (ChEBI)
MECR ProteinQ9BV79 (Uniprot-TrEMBL)
MECR dimerComplexR-HSA-8952866 (Reactome)
MEMA-CoAMetaboliteCHEBI:15466 (ChEBI)
MMAA ProteinQ8IVH4 (Uniprot-TrEMBL)
MUT ProteinP22033 (Uniprot-TrEMBL)
MYS-CoAMetaboliteCHEBI:15532 (ChEBI)
Mal-PPANT-S112-NDUFAB1ProteinO14561 (Uniprot-TrEMBL)
Malonyl-CoAMetaboliteCHEBI:15531 (ChEBI)
NAD+MetaboliteCHEBI:57540 (ChEBI)
NADHMetaboliteCHEBI:57945 (ChEBI)
NADP+MetaboliteCHEBI:18009 (ChEBI)
NADPHMetaboliteCHEBI:16474 (ChEBI)
Octanoyl-CoAMetaboliteCHEBI:15533 (ChEBI)
PCCB ProteinP05166 (Uniprot-TrEMBL)
PCTPProteinQ9UKL6 (Uniprot-TrEMBL)
PPANT-S112-NDUFAB1ProteinO14561 (Uniprot-TrEMBL)
PPiMetaboliteCHEBI:29888 (ChEBI)
PROP-CoAMetaboliteCHEBI:15539 (ChEBI)
PalmCoAMetaboliteCHEBI:15525 (ChEBI)
PiMetaboliteCHEBI:43474 (ChEBI)
S-2MDPDA-CoAMetaboliteCHEBI:85945 (ChEBI)
S-2MPDA-CoAMetaboliteCHEBI:85944 (ChEBI)
SUCC-CoAMetaboliteCHEBI:57292 (ChEBI)
THEM4 ProteinQ5T1C6 (Uniprot-TrEMBL)
THEM5 ProteinQ8N1Q8 (Uniprot-TrEMBL)
Trifunctional ProteinComplexR-HSA-77267 (Reactome)
acyl-CoAMetaboliteCHEBI:17984 (ChEBI)
adenosine 5'-monophosphateMetaboliteCHEBI:16027 (ChEBI)
cis,cis-3,6-Dodecadienoyl-CoAMetaboliteCHEBI:28002 (ChEBI)
tdec2-CoAMetaboliteCHEBI:10723 (ChEBI)
trans,cis-Lauro-2,6-dienoyl-CoAMetaboliteCHEBI:28387 (ChEBI)
trans-Hex-2-enoyl-CoAMetaboliteCHEBI:28706 (ChEBI)
trans-Hexadec-2-enoyl-CoAMetaboliteCHEBI:28935 (ChEBI)
trans-Oct-2-enoyl-CoAMetaboliteCHEBI:27537 (ChEBI)
trans-Tetradec-2-enoyl-CoAMetaboliteCHEBI:27721 (ChEBI)

Annotated Interactions

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SourceTargetTypeDatabase referenceComment
(S)-3-Hydroxydodecanoyl-CoAArrowR-HSA-77256 (Reactome)
(S)-3-Hydroxydodecanoyl-CoAR-HSA-77254 (Reactome)
(S)-3-Hydroxyhexadecanoyl-CoAArrowR-HSA-77301 (Reactome)
(S)-3-Hydroxyhexadecanoyl-CoAR-HSA-77303 (Reactome)
(S)-3-Hydroxytetradecanoyl-CoAArrowR-HSA-77277 (Reactome)
(S)-3-Hydroxytetradecanoyl-CoAR-HSA-77283 (Reactome)
(S)-Hydroxydecanoyl-CoAArrowR-HSA-77344 (Reactome)
(S)-Hydroxydecanoyl-CoAR-HSA-77342 (Reactome)
(S)-Hydroxyhexanoyl-CoAArrowR-HSA-77325 (Reactome)
(S)-Hydroxyhexanoyl-CoAR-HSA-77323 (Reactome)
(S)-Hydroxyoctanoyl-CoAArrowR-HSA-77333 (Reactome)
(S)-Hydroxyoctanoyl-CoAR-HSA-77331 (Reactome)
2-trans-4-cis-decadienoyl-CoAArrowR-HSA-109341 (Reactome)
2-trans-4-cis-decadienoyl-CoAR-HSA-109343 (Reactome)
2-trans-Dodecenoyl-CoAArrowR-HSA-77263 (Reactome)
2-trans-Dodecenoyl-CoAR-HSA-77256 (Reactome)
2xMMAA:2xMUT:AdoCblmim-catalysisR-HSA-71010 (Reactome)
3-Oxodecanoyl-CoAArrowR-HSA-77342 (Reactome)
3-Oxodecanoyl-CoAR-HSA-77340 (Reactome)
3-Oxododecanoyl-CoAArrowR-HSA-77254 (Reactome)
3-Oxododecanoyl-CoAR-HSA-77309 (Reactome)
3-Oxohexanoyl-CoAArrowR-HSA-77323 (Reactome)
3-Oxohexanoyl-CoAR-HSA-77321 (Reactome)
3-Oxooctanoyl-CoAArrowR-HSA-77331 (Reactome)
3-Oxooctanoyl-CoAR-HSA-77329 (Reactome)
3-Oxopalmitoyl-CoAArrowR-HSA-77303 (Reactome)
3-Oxopalmitoyl-CoAR-HSA-77304 (Reactome)
3-Oxotetradecanoyl-CoAArrowR-HSA-77283 (Reactome)
3-Oxotetradecanoyl-CoAR-HSA-77271 (Reactome)
3-trans-decenoyl-CoAArrowR-HSA-109343 (Reactome)
3-trans-decenoyl-CoAR-HSA-109998 (Reactome)
3HB-CoAArrowR-HSA-77314 (Reactome)
3HB-CoAR-HSA-77312 (Reactome)
3OA-CoAArrowR-HSA-8874745 (Reactome)
4-cis-decenoyl-CoAArrowR-HSA-109342 (Reactome)
4-cis-decenoyl-CoAR-HSA-109341 (Reactome)
6x(Btn-PCCA:PCCB)mim-catalysisR-HSA-71031 (Reactome)
ACA-CoAArrowR-HSA-77312 (Reactome)
ACAA2 tetramermim-catalysisR-HSA-8874745 (Reactome)
ACAD10mim-catalysisR-HSA-5695980 (Reactome)
ACAD11mim-catalysisR-HSA-5695989 (Reactome)
ACADL tetramerArrowR-HSA-109339 (Reactome)
ACADL tetramerArrowR-HSA-109342 (Reactome)
ACADL tetramermim-catalysisR-HSA-77263 (Reactome)
ACADL tetramermim-catalysisR-HSA-77274 (Reactome)
ACADM tetramermim-catalysisR-HSA-109341 (Reactome)
ACADM tetramermim-catalysisR-HSA-77338 (Reactome)
ACADM tetramermim-catalysisR-HSA-77345 (Reactome)
ACADS tetramermim-catalysisR-HSA-77319 (Reactome)
ACADS tetramermim-catalysisR-HSA-77327 (Reactome)
ACADVL dimermim-catalysisR-HSA-77299 (Reactome)
ACBD6:LCFA-CoAArrowR-HSA-8848250 (Reactome)
ACBD6R-HSA-8848250 (Reactome)
ACOT2,9, THEM4,5 dimermim-catalysisR-HSA-5690066 (Reactome)
ACSF2mim-catalysisR-HSA-5696004 (Reactome)
ACSM3,ACSM6mim-catalysisR-HSA-8875013 (Reactome)
ADPArrowR-HSA-71031 (Reactome)
ATPR-HSA-5696004 (Reactome)
ATPR-HSA-71031 (Reactome)
ATPR-HSA-8875013 (Reactome)
Ac-CoAArrowR-HSA-109339 (Reactome)
Ac-CoAArrowR-HSA-109342 (Reactome)
Ac-CoAArrowR-HSA-77271 (Reactome)
Ac-CoAArrowR-HSA-77304 (Reactome)
Ac-CoAArrowR-HSA-77309 (Reactome)
Ac-CoAArrowR-HSA-77321 (Reactome)
Ac-CoAArrowR-HSA-77329 (Reactome)
Ac-CoAArrowR-HSA-77340 (Reactome)
Ac-CoAR-HSA-8874745 (Reactome)
BH-CoAR-HSA-5695989 (Reactome)
BT-CoAArrowR-HSA-77321 (Reactome)
BT-CoAArrowR-HSA-8875013 (Reactome)
BT-CoAR-HSA-77319 (Reactome)
BUTR-HSA-8875013 (Reactome)
CO2R-HSA-71031 (Reactome)
CoA-SHArrowR-HSA-5690043 (Reactome)
CoA-SHArrowR-HSA-5690066 (Reactome)
CoA-SHArrowR-HSA-8874745 (Reactome)
CoA-SHArrowR-HSA-8933547 (Reactome)
CoA-SHR-HSA-109339 (Reactome)
CoA-SHR-HSA-109342 (Reactome)
CoA-SHR-HSA-5696004 (Reactome)
CoA-SHR-HSA-77271 (Reactome)
CoA-SHR-HSA-77304 (Reactome)
CoA-SHR-HSA-77309 (Reactome)
CoA-SHR-HSA-77321 (Reactome)
CoA-SHR-HSA-77329 (Reactome)
CoA-SHR-HSA-77340 (Reactome)
Crotonoyl-CoAArrowR-HSA-77319 (Reactome)
Crotonoyl-CoAR-HSA-77314 (Reactome)
Cytosolic ACOTs, THEM4mim-catalysisR-HSA-5690043 (Reactome)
DBI, (ACBD7)R-HSA-8848246 (Reactome)
DBI,ACBD7:MCFA-CoA,LCFA-CoAArrowR-HSA-8848246 (Reactome)
DCI dimermim-catalysisR-HSA-109338 (Reactome)
DCI dimermim-catalysisR-HSA-109998 (Reactome)
DEC-CoAArrowR-HSA-77309 (Reactome)
DEC-CoAArrowR-HSA-8952873 (Reactome)
DEC-CoAR-HSA-77345 (Reactome)
DECR1 tetramermim-catalysisR-HSA-109343 (Reactome)
DHB-CoAArrowR-HSA-5695989 (Reactome)
ECHS1 hexamermim-catalysisR-HSA-77256 (Reactome)
ECHS1 hexamermim-catalysisR-HSA-77314 (Reactome)
ECHS1 hexamermim-catalysisR-HSA-77325 (Reactome)
ECHS1 hexamermim-catalysisR-HSA-77333 (Reactome)
ECHS1 hexamermim-catalysisR-HSA-77344 (Reactome)
FADH2ArrowR-HSA-109339 (Reactome)
FADH2ArrowR-HSA-109341 (Reactome)
FADH2ArrowR-HSA-109342 (Reactome)
FADH2ArrowR-HSA-5695980 (Reactome)
FADH2ArrowR-HSA-5695989 (Reactome)
FADH2ArrowR-HSA-77263 (Reactome)
FADH2ArrowR-HSA-77274 (Reactome)
FADH2ArrowR-HSA-77299 (Reactome)
FADH2ArrowR-HSA-77319 (Reactome)
FADH2ArrowR-HSA-77327 (Reactome)
FADH2ArrowR-HSA-77338 (Reactome)
FADH2ArrowR-HSA-77345 (Reactome)
FADR-HSA-109339 (Reactome)
FADR-HSA-109341 (Reactome)
FADR-HSA-109342 (Reactome)
FADR-HSA-5695980 (Reactome)
FADR-HSA-5695989 (Reactome)
FADR-HSA-77263 (Reactome)
FADR-HSA-77274 (Reactome)
FADR-HSA-77299 (Reactome)
FADR-HSA-77319 (Reactome)
FADR-HSA-77327 (Reactome)
FADR-HSA-77338 (Reactome)
FADR-HSA-77345 (Reactome)
H+ArrowR-HSA-109339 (Reactome)
H+ArrowR-HSA-109342 (Reactome)
H+ArrowR-HSA-77254 (Reactome)
H+ArrowR-HSA-77283 (Reactome)
H+ArrowR-HSA-77303 (Reactome)
H+ArrowR-HSA-77312 (Reactome)
H+ArrowR-HSA-77323 (Reactome)
H+ArrowR-HSA-77331 (Reactome)
H+ArrowR-HSA-77342 (Reactome)
H+R-HSA-109343 (Reactome)
H+R-HSA-8952873 (Reactome)
H2OR-HSA-109339 (Reactome)
H2OR-HSA-109342 (Reactome)
H2OR-HSA-5690043 (Reactome)
H2OR-HSA-5690066 (Reactome)
H2OR-HSA-77256 (Reactome)
H2OR-HSA-77277 (Reactome)
H2OR-HSA-77301 (Reactome)
H2OR-HSA-77314 (Reactome)
H2OR-HSA-77325 (Reactome)
H2OR-HSA-77333 (Reactome)
H2OR-HSA-77344 (Reactome)
HADH dimermim-catalysisR-HSA-77254 (Reactome)
HADH dimermim-catalysisR-HSA-77312 (Reactome)
HADH dimermim-catalysisR-HSA-77323 (Reactome)
HADH dimermim-catalysisR-HSA-77331 (Reactome)
HADH dimermim-catalysisR-HSA-77342 (Reactome)
HX-CoAArrowR-HSA-77329 (Reactome)
HX-CoAR-HSA-77327 (Reactome)
L-MM-CoAArrowR-HSA-71020 (Reactome)
L-MM-CoAR-HSA-71010 (Reactome)
LAU-CoAArrowR-HSA-77271 (Reactome)
LAU-CoAR-HSA-77263 (Reactome)
LCFA-CoAR-HSA-8848250 (Reactome)
LIN-CoAR-HSA-109339 (Reactome)
MCATmim-catalysisR-HSA-8933547 (Reactome)
MCEEmim-catalysisR-HSA-71020 (Reactome)
MCFA, LCFAArrowR-HSA-5690043 (Reactome)
MCFA, LCFAArrowR-HSA-5690066 (Reactome)
MCFA-CoA, LCFA-CoAR-HSA-5690043 (Reactome)
MCFA-CoA, LCFA-CoAR-HSA-5690066 (Reactome)
MCFA-CoA, LCFA-CoAR-HSA-8848246 (Reactome)
MCFA-CoAArrowR-HSA-5696004 (Reactome)
MCFAR-HSA-5696004 (Reactome)
MECR dimermim-catalysisR-HSA-8952873 (Reactome)
MEMA-CoAArrowR-HSA-71031 (Reactome)
MEMA-CoAR-HSA-71020 (Reactome)
MYS-CoAArrowR-HSA-77304 (Reactome)
MYS-CoAR-HSA-77274 (Reactome)
Mal-PPANT-S112-NDUFAB1ArrowR-HSA-8933547 (Reactome)
Malonyl-CoAR-HSA-8933547 (Reactome)
NAD+ArrowR-HSA-109343 (Reactome)
NAD+R-HSA-109339 (Reactome)
NAD+R-HSA-109342 (Reactome)
NAD+R-HSA-77254 (Reactome)
NAD+R-HSA-77283 (Reactome)
NAD+R-HSA-77303 (Reactome)
NAD+R-HSA-77312 (Reactome)
NAD+R-HSA-77323 (Reactome)
NAD+R-HSA-77331 (Reactome)
NAD+R-HSA-77342 (Reactome)
NADHArrowR-HSA-109339 (Reactome)
NADHArrowR-HSA-109342 (Reactome)
NADHArrowR-HSA-77254 (Reactome)
NADHArrowR-HSA-77283 (Reactome)
NADHArrowR-HSA-77303 (Reactome)
NADHArrowR-HSA-77312 (Reactome)
NADHArrowR-HSA-77323 (Reactome)
NADHArrowR-HSA-77331 (Reactome)
NADHArrowR-HSA-77342 (Reactome)
NADHR-HSA-109343 (Reactome)
NADP+ArrowR-HSA-8952873 (Reactome)
NADPHR-HSA-8952873 (Reactome)
Octanoyl-CoAArrowR-HSA-77340 (Reactome)
Octanoyl-CoAR-HSA-77338 (Reactome)
PCTPArrowR-HSA-5690043 (Reactome)
PPANT-S112-NDUFAB1R-HSA-8933547 (Reactome)
PPiArrowR-HSA-5696004 (Reactome)
PPiArrowR-HSA-8875013 (Reactome)
PROP-CoAR-HSA-71031 (Reactome)
PalmCoAR-HSA-77299 (Reactome)
PiArrowR-HSA-71031 (Reactome)
R-HSA-109338 (Reactome) At the beginning of this reaction, 1 molecule of 'cis,cis-3,6-Dodecadienoyl-CoA' is present. At the end of this reaction, 1 molecule of 'trans,cis-Lauro-2,6-dienoyl-CoA ' is present.

This reaction takes place in the 'mitochondrial matrix' and is mediated by the 'dodecenoyl-CoA delta-isomerase activity' of '3,2-trans-enoyl-CoA isomerase Homodimer'.

R-HSA-109339 (Reactome) Three cycles of beta oxidation, each mediated by the activities of trifunctional protein and ACADL tetramer, convert linoleoyl-CoA to cis,cis-3,6-dodecadienoyl-CoA plus three molecules each of CoA-SH, FADH2, and NADH + H+.
R-HSA-109341 (Reactome) 4-cis-decenoyl-CoA transits through the first step of the saturated beta-oxidation spiral to yield 2-trans-4-cis-decadienoyl-CoA.
R-HSA-109342 (Reactome) One cycle of beta oxidation, each mediated by the activities of trifunctional protein and ACADL tetramer, converts trans,cis-lauro-2,6-dienoyl-CoA to 4-cis-decenoyl-CoA plus molecules of CoA-SH, FADH2, and NADH + H+.
R-HSA-109343 (Reactome) The second of the two accessory enzymes, 2,4-dienoyl-CoA reductase catalyses an oxidation-reduction (redox) reaction to yield 3-trans-decenoyl-CoA.
R-HSA-109998 (Reactome) Once the second of the two double bonds has been reached 3,2-trans-enoyl-CoA isomerase, changes the spatial conformation of the second double bond from cis to trans. This step yields trans-dec-2-enoyl-CoA, which then enters the saturated beta-oxidation pathway.
R-HSA-5690043 (Reactome) The maintenance/regulation of cellular levels of free fatty acids and fatty acyl-CoAs (the activated form of free fatty acids) is extremely important, as imbalances in lipid metabolism can have serious consequences for human health. Free fatty acids can act as detergents to disrupt membranes so their generation is normally tightly regulated to states where they will be rapidly consumed or sequestered. Acyl-coenzyme A thioesterases (ACOTs) hydrolyse the thioester bond in medium- to long-chain fatty acyl-CoAs (of C12-C18 lengths) (MCFA-CoA, LCFA-CoA) to their free fatty acids (MCFA, LCFA) (Cohen 2013, Hunt et al. 2012, Kirkby et al. 2010). ACOTs that function in the cytosol are ACOT1 (Hunt et al. 2005), ACOT11 (Adams et al. 2001), ACOT12 trimer (Swarbrick et al. 2014), ACOT13 tetramer (Cao et al. 2009, Cheng et al. 2006), ACOT7 hexamer (Hunt et al. 2005b) and ACOT7L dimer (Jiang et al. 2006).

Recent mouse studies reveals a key regulatory role for PCTP in lipid and glucose metabolism. Phosphatidylcholine transfer protein (PCTP aka STARD2) is a member of the steroidogenic acute regulatory protein (StAR)-related lipid transfer (START) domain superfamily, a functionally diverse group of proteins that share a unique structural motif for binding lipids. PCTP appears to limit access of fatty acids to mitochondria by binding to (Ersoy et al. 2013) and stimulating the activity of acyl-coenzyme A thioesterase 13 (ACOT13, aka Acyl-CoA thioesterase 13, THEM2), an enzyme that catalyses the hydrolysis of acyl-CoAs to their free fatty acids (Kawano et al. 2014). Ultimately, insulin signaling is downregulated (Kang et al. 2010).
R-HSA-5690066 (Reactome) The maintenance/regulation of cellular levels of free fatty acids and fatty acyl-CoAs (the activated form of free fatty acids) is extremely important, as imbalances in lipid metabolism can have serious consequences for human health. Acyl-coenzyme A thioesterases (ACOTs) hydrolyse the thioester bond in medium- to long-chain fatty acyl-CoAs (of C12-C18 lengths) (MCFA-CoA, LCFA-CoA) to their free fatty acids (MCFA, LCFA) (Cohen 2013, Hunt et al. 2012, Kirkby et al. 2010). ACOTs that function in the mitochondrion are ACOT2 (Hunt et al. 2006), ACOT9 (Kirkby et al. 2010), THEM4 dimer (Zhuravleva et al. 2012, Zhao et al. 2012) and THEM5 dimer (Zhuravleva et al. 2012). THEM4 is also functional in the cytosol and at the plasma membrane (Cohen 2013).
R-HSA-5695980 (Reactome) Acyl-CoA dehydrogenase family member 10 (ACAD10) is a mitochondrial enzyme that can catalyse the alpha, beta-dehydrogenation of acyl-CoA esters. ACAD10 shows highest expression in foetal brain and is shown to be active only on S-2-methylpentadecenoyl-CoA (S-2MPDA-CoA), a C15 acyl-CoA. The S isomer is dehydrogenated to its respective 2,3-dehydroacyl-CoA product, S-2methyl-2,3-dehydropentadecenoyl-CoA (S-2MDPDA) (He et al. 2011).
R-HSA-5695989 (Reactome) Acyl-CoA dehydrogenase family member 11 (ACAD11) is a mitochondrial membrane-bound enzyme that can catalyse the alpha, beta-dehydrogenation of acyl-CoA esters. ACAD11 shows highest expression in the brain and is shown to dehydrogenate the C22 acyl-CoA behenoyl-CoA (BH-CoA) to 2,3-dehydrobehenoyl-CoA (DBH-CoA) (He et al. 2011).
R-HSA-5696004 (Reactome) Acyl-coenzyme A synthetases (ACSs) catalyse the activation of fatty acids by thioesterification to CoA, the fundamental initial reaction in fatty acid metabolism. Mitochondrial acyl-CoA synthetase family member 2 (ACSF2) preferentially ligates CoA-SH to medium-chain fatty acids (MCFA), around C8 in length (Watkins et al. 2007).
R-HSA-71010 (Reactome) Methylmalonyl CoA mutase (MUT aka MCM) (Jansen et al. 1989) utilises adenosylcobalamin (AdoCbl) as a cofactor and catalyzes interchange of a carbonyl-CoA group and a hydrogen atom in conversion of methylmalonyl CoA to form succinyl CoA, a precursor for the citric acid cycle. MUT has a homodimeric structure and is located in the mitochondrial matrix. Defects in MUT cause methylmalonic aciduria, mut type (MMAM; MIM:251000), an often fatal disorder of organic acid metabolism (Worgan et al. 2006).

Methylmalonic aciduria type A protein (MMAA) is thought to act as a chaperone to MUT, the enzyme which utilises adenosylcobalamin (AdoCbl) as a cofactor. MMAA is suggested to play a dual role with regards to MUT protection and reactivation. Some AdoCbl-dependent enzymes undergo suicide inactivation after catalysis due to the oxidative inactivation of Cbl. MMAA is thought to play a protective role to prevent MUT being inactivated in this way. After the catalytic cycle when MUT is inactive, MMAA increases the enzymatic activity of MUT through exchange of the damaged cofactor. Whether this happens via GTP-mediated hydrolysis is unknown at present (Takahashi-Iniguez et al. 2011, Froese et al. 2010). Bacterial AdoCbl-containing enzymes possess reactivating factors which release the inactivated cofactor to allow the resulting apoenzyme to reconstitute into an active form. A bacterial orthologue of MMAA, MeaB, forms a stable complex with MUT and plays a role in its protection and reactivation (Padovani & Banerjee 2006).

Defects in MMAA cause methylmalonic aciduria type cblA (cblA aka methylmalonic aciduria type A or vitamin B12-responsive methylmalonicaciduria of cblA complementation type; MIM:251100). Affected individuals accumulate methylmalonic acid in the blood and urine and are prone to potentially life threatening acidotic crises in infancy or early childhood (Dobson et al. 2002, Lerner-Ellis et al. 2004).
R-HSA-71020 (Reactome) At the beginning of this reaction, 1 molecule of 'D-methylmalonyl-CoA' is present. At the end of this reaction, 1 molecule of 'L-methylmalonyl-CoA' is present.

This reaction takes place in the 'mitochondrial matrix' and is mediated by the 'methylmalonyl-CoA epimerase activity' of 'methylmalonyl-CoA epimerase'.

R-HSA-71031 (Reactome) Propionyl CoA carboxylase in the mitochondrial matrix catalyzes the reaction of propionyl-CoA, CO2, and ATP to form D-methylmalonyl-CoA, ADP, and orthophosphate. The active form of the enzyme is a heteromultimer, probably consisting of six alpha subunits each bound to a biotin molecule and six beta subunits (Kaziro et al. 1961; Kalousek et al. 1980; Fenton et al. 2001). Both alpha and beta subunits are posttranslationally modified to remove amino-terminal mitochondrial import sequences (Stadler et al. 2005).
R-HSA-77254 (Reactome) At the beginning of this reaction, 1 molecule of '(S)-3-Hydroxydodecanoyl-CoA', and 1 molecule of 'NAD+' are present. At the end of this reaction, 1 molecule of 'H+', 1 molecule of '3-Oxododecanoyl-CoA', and 1 molecule of 'NADH' are present.

This reaction takes place in the 'mitochondrial matrix' and is mediated by the '3-hydroxyacyl-CoA dehydrogenase activity' of 'short chain 3-hydroxyacyl-CoA dehydrogenase homodimer'.

R-HSA-77256 (Reactome) At the beginning of this reaction, 1 molecule of '2-trans-Dodecenoyl-CoA', and 1 molecule of 'H2O' are present. At the end of this reaction, 1 molecule of '(S)-3-Hydroxydodecanoyl-CoA' is present.

This reaction takes place in the 'mitochondrial matrix' and is mediated by the 'enoyl-CoA hydratase activity' of 'enoyl-CoA hydratase hexamer'.

R-HSA-77263 (Reactome) At the beginning of this reaction, 1 molecule of 'Lauroyl-CoA', and 1 molecule of 'FAD' are present. At the end of this reaction, 1 molecule of 'FADH2', and 1 molecule of '2-trans-Dodecenoyl-CoA' are present.

This reaction takes place in the 'mitochondrial matrix' and is mediated by the 'acyl-CoA dehydrogenase activity' of 'LCAD acyl-CoA dehydrogenase homotetramer'.

R-HSA-77271 (Reactome) At the beginning of this reaction, 1 molecule of '3-Oxotetradecanoyl-CoA', and 1 molecule of 'CoA' are present. At the end of this reaction, 1 molecule of 'Lauroyl-CoA', and 1 molecule of 'Acetyl-CoA' are present.

This reaction takes place in the 'mitochondrial matrix' and is mediated by the 'transferase activity' of 'Trifunctional Protein'.

R-HSA-77274 (Reactome) At the beginning of this reaction, 1 molecule of 'myristoyl-CoA', and 1 molecule of 'FAD' are present. At the end of this reaction, 1 molecule of 'FADH2', and 1 molecule of 'trans-Tetradec-2-enoyl-CoA' are present.

This reaction takes place in the 'mitochondrial matrix' and is mediated by the 'acyl-CoA dehydrogenase activity' of 'LCAD acyl-CoA dehydrogenase homotetramer'.

R-HSA-77277 (Reactome) At the beginning of this reaction, 1 molecule of 'trans-Tetradec-2-enoyl-CoA', and 1 molecule of 'H2O' are present. At the end of this reaction, 1 molecule of '(S)-3-Hydroxytetradecanoyl-CoA' is present.

This reaction takes place in the 'mitochondrial matrix' and is mediated by the 'enoyl-CoA hydratase activity' of 'Trifunctional Protein'.

R-HSA-77283 (Reactome) At the beginning of this reaction, 1 molecule of 'NAD+', and 1 molecule of '(S)-3-Hydroxytetradecanoyl-CoA' are present. At the end of this reaction, 1 molecule of 'H+', 1 molecule of '3-Oxotetradecanoyl-CoA', and 1 molecule of 'NADH' are present.

This reaction takes place in the 'mitochondrial matrix' and is mediated by the '3-hydroxyacyl-CoA dehydrogenase activity' of 'Trifunctional Protein'.

R-HSA-77299 (Reactome) At the beginning of this reaction, 1 molecule of 'palmitoyl-CoA', and 1 molecule of 'FAD' are present. At the end of this reaction, 1 molecule of 'FADH2', and 1 molecule of 'trans-Hexadec-2-enoyl-CoA' are present.

This reaction takes place in the 'mitochondrial matrix' and is mediated by the 'acyl-CoA dehydrogenase activity' of 'VLCAD acyl-CoA dehydrogenase homodimer'.

R-HSA-77301 (Reactome) At the beginning of this reaction, 1 molecule of 'H2O', and 1 molecule of 'trans-Hexadec-2-enoyl-CoA' are present. At the end of this reaction, 1 molecule of '(S)-3-Hydroxyhexadecanoyl-CoA' is present.

This reaction takes place in the 'mitochondrial matrix' and is mediated by the 'enoyl-CoA hydratase activity' of 'Trifunctional Protein'.

R-HSA-77303 (Reactome) At the beginning of this reaction, 1 molecule of 'NAD+', and 1 molecule of '(S)-3-Hydroxyhexadecanoyl-CoA' are present. At the end of this reaction, 1 molecule of '3-Oxopalmitoyl-CoA', 1 molecule of 'H+', and 1 molecule of 'NADH' are present.

This reaction takes place in the 'mitochondrial matrix' and is mediated by the '3-hydroxyacyl-CoA dehydrogenase activity' of 'Trifunctional Protein'.

R-HSA-77304 (Reactome) At the beginning of this reaction, 1 molecule of '3-Oxopalmitoyl-CoA', and 1 molecule of 'CoA' are present. At the end of this reaction, 1 molecule of 'Acetyl-CoA', and 1 molecule of 'myristoyl-CoA' are present.

This reaction takes place in the 'mitochondrial matrix' and is mediated by the 'transferase activity' of 'Trifunctional Protein'.

R-HSA-77309 (Reactome) At the beginning of this reaction, 1 molecule of '3-Oxododecanoyl-CoA', and 1 molecule of 'CoA' are present. At the end of this reaction, 1 molecule of 'Decanoyl-CoA', and 1 molecule of 'Acetyl-CoA' are present.

This reaction takes place in the 'mitochondrial matrix' and is mediated by the 'transferase activity' of 'Trifunctional Protein'.

R-HSA-77312 (Reactome) At the beginning of this reaction, 1 molecule of 'NAD+', and 1 molecule of '(S)-3-Hydroxybutanoyl-CoA' are present. At the end of this reaction, 1 molecule of 'acetoacetyl-CoA', 1 molecule of 'H+', and 1 molecule of 'NADH' are present.

This reaction takes place in the 'mitochondrial matrix' and is mediated by the '3-hydroxyacyl-CoA dehydrogenase activity' of 'short chain 3-hydroxyacyl-CoA dehydrogenase homodimer'.

R-HSA-77314 (Reactome) At the beginning of this reaction, 1 molecule of 'Crotonoyl-CoA', and 1 molecule of 'H2O' are present. At the end of this reaction, 1 molecule of '(S)-3-Hydroxybutanoyl-CoA' is present.

This reaction takes place in the 'mitochondrial matrix' and is mediated by the 'enoyl-CoA hydratase activity' of 'enoyl-CoA hydratase hexamer'.

R-HSA-77319 (Reactome) At the beginning of this reaction, 1 molecule of 'FAD', and 1 molecule of 'Butanoyl-CoA' are present. At the end of this reaction, 1 molecule of 'Crotonoyl-CoA', and 1 molecule of 'FADH2' are present.

This reaction takes place in the 'mitochondrial matrix' and is mediated by the 'acyl-CoA dehydrogenase activity' of 'SCAD acyl-CoA dehydrogenase homotetramer'.

R-HSA-77321 (Reactome) At the beginning of this reaction, 1 molecule of '3-Oxohexanoyl-CoA', and 1 molecule of 'CoA' are present. At the end of this reaction, 1 molecule of 'Acetyl-CoA', and 1 molecule of 'Butanoyl-CoA' are present.

This reaction takes place in the 'mitochondrial matrix' and is mediated by the 'transferase activity' of 'Trifunctional Protein'.

R-HSA-77323 (Reactome) At the beginning of this reaction, 1 molecule of 'NAD+', and 1 molecule of '(S)-Hydroxyhexanoyl-CoA' are present. At the end of this reaction, 1 molecule of '3-Oxohexanoyl-CoA', 1 molecule of 'H+', and 1 molecule of 'NADH' are present.

This reaction takes place in the 'mitochondrial matrix' and is mediated by the '3-hydroxyacyl-CoA dehydrogenase activity' of 'short chain 3-hydroxyacyl-CoA dehydrogenase homodimer'.

R-HSA-77325 (Reactome) At the beginning of this reaction, 1 molecule of 'trans-Hex-2-enoyl-CoA', and 1 molecule of 'H2O' are present. At the end of this reaction, 1 molecule of '(S)-Hydroxyhexanoyl-CoA' is present.

This reaction takes place in the 'mitochondrial matrix' and is mediated by the 'enoyl-CoA hydratase activity' of 'enoyl-CoA hydratase hexamer'.

R-HSA-77327 (Reactome) At the beginning of this reaction, 1 molecule of 'Hexanoyl-CoA', and 1 molecule of 'FAD' are present. At the end of this reaction, 1 molecule of 'trans-Hex-2-enoyl-CoA', and 1 molecule of 'FADH2' are present.

This reaction takes place in the 'mitochondrial matrix' and is mediated by the 'acyl-CoA dehydrogenase activity' of 'SCAD acyl-CoA dehydrogenase homotetramer'.

R-HSA-77329 (Reactome) At the beginning of this reaction, 1 molecule of '3-Oxooctanoyl-CoA', and 1 molecule of 'CoA' are present. At the end of this reaction, 1 molecule of 'Hexanoyl-CoA', and 1 molecule of 'Acetyl-CoA' are present.

This reaction takes place in the 'mitochondrial matrix' and is mediated by the 'transferase activity' of 'Trifunctional Protein'.

R-HSA-77331 (Reactome) At the beginning of this reaction, 1 molecule of 'NAD+', and 1 molecule of '(S)-Hydroxyoctanoyl-CoA' are present. At the end of this reaction, 1 molecule of '3-Oxooctanoyl-CoA', 1 molecule of 'H+', and 1 molecule of 'NADH' are present.

This reaction takes place in the 'mitochondrial matrix' and is mediated by the '3-hydroxyacyl-CoA dehydrogenase activity' of 'short chain 3-hydroxyacyl-CoA dehydrogenase homodimer'.

R-HSA-77333 (Reactome) At the beginning of this reaction, 1 molecule of 'H2O', and 1 molecule of 'trans-Oct-2-enoyl-CoA' are present. At the end of this reaction, 1 molecule of '(S)-Hydroxyoctanoyl-CoA' is present.

This reaction takes place in the 'mitochondrial matrix' and is mediated by the 'enoyl-CoA hydratase activity' of 'enoyl-CoA hydratase hexamer'.

R-HSA-77338 (Reactome) At the beginning of this reaction, 1 molecule of 'Octanoyl-CoA', and 1 molecule of 'FAD' are present. At the end of this reaction, 1 molecule of 'FADH2', and 1 molecule of 'trans-Oct-2-enoyl-CoA' are present.

This reaction takes place in the 'mitochondrial matrix' and is mediated by the 'acyl-CoA dehydrogenase activity' of 'MCAD acyl-CoA dehydrogenase homotetramer'.

R-HSA-77340 (Reactome) At the beginning of this reaction, 1 molecule of '3-Oxodecanoyl-CoA', and 1 molecule of 'CoA' are present. At the end of this reaction, 1 molecule of 'Acetyl-CoA', and 1 molecule of 'Octanoyl-CoA' are present.

This reaction takes place in the 'mitochondrial matrix' and is mediated by the 'transferase activity' of 'Trifunctional Protein'.

R-HSA-77342 (Reactome) At the beginning of this reaction, 1 molecule of '(S)-Hydroxydecanoyl-CoA', and 1 molecule of 'NAD+' are present. At the end of this reaction, 1 molecule of 'H+', 1 molecule of '3-Oxodecanoyl-CoA', and 1 molecule of 'NADH' are present.

This reaction takes place in the 'mitochondrial matrix' and is mediated by the '3-hydroxyacyl-CoA dehydrogenase activity' of 'short chain 3-hydroxyacyl-CoA dehydrogenase homodimer'.

R-HSA-77344 (Reactome) At the beginning of this reaction, 1 molecule of 'trans-Dec-2-enoyl-CoA', and 1 molecule of 'H2O' are present. At the end of this reaction, 1 molecule of '(S)-Hydroxydecanoyl-CoA' is present.

This reaction takes place in the 'mitochondrial matrix' and is mediated by the 'enoyl-CoA hydratase activity' of 'enoyl-CoA hydratase hexamer'.

R-HSA-77345 (Reactome) At the beginning of this reaction, 1 molecule of 'Decanoyl-CoA', and 1 molecule of 'FAD' are present. At the end of this reaction, 1 molecule of 'trans-Dec-2-enoyl-CoA', and 1 molecule of 'FADH2' are present.

This reaction takes place in the 'mitochondrial matrix' and is mediated by the 'acyl-CoA dehydrogenase activity' of 'MCAD acyl-CoA dehydrogenase homotetramer'.

R-HSA-8848246 (Reactome) Acyl-CoA-binding protein (DBI, aka ACBP) can bind medium- and long-chain acyl-CoA esters (MCFA-CoA, LCFA-CoA) with very high affinity. It is localised to the ER (and Golgi) and may function as an intracellular carrier of acyl-CoA esters (Hansen et al. 2008, Bloksgaard et al. 2014). Acyl-CoA-binding domain-containing protein 7 (ACBD7) shares around 60% sequence homology with DBI and is proposed to also bind fatty acyl-CoAs but its function is yet to be determined.
R-HSA-8848250 (Reactome) Acyl-CoA-binding domain-containing protein 6 (ACBD6) has an acyl-CoA binding domain at its N terminus and two ankyrin motifs at its C terminus. ACBD6 binds long-chain acyl-CoAs (LCFA-CoA) with a strong preference for unsaturated, C18:1-CoA and C20:4-CoA, over saturated, C16:0-CoA substrates. ACBD6 is expressed in tissues and progenitor cells with functions in blood and vessel development (Soupene et al. 2008). A possible role of ACBD6 could be to protect membrane systems from the detergent nature of free acyl-CoAs by controlling their release to acyl-CoA-utilising enzymes (Soupene & Kuypers 2015).
R-HSA-8874745 (Reactome) Mitochondrial 3-ketoacyl-CoA thiolase (ACAA2) is a mitochondrial matrix enzyme involved in fatty acid beta-oxidation, transferring the acyl group from acyl-CoA (acyl-CoA) to acetyl-CoA (Ac-CoA) to form 3-oxyoacyl-CoA (3OA-CoA) and CoA-SH (Abe et al. 1993, Middleton 1973).
R-HSA-8875013 (Reactome) Mitochondrial acyl-coenzyme A synthetase ACSM3 (aka protein SAH homolog) is expressed in the mitochondrial matrix and possesses medium-chain fatty acid:CoA ligase activity. Based on characterisation experiments in mice, ACSM3 preferentially ligates C2-C6 fatty acids, especially butyrate (BUT, a C4 fatty acid) (Fujino et al. 2001). The product, butyryl-CoA (BT-CoA) is used in beta oxidation. ACSM3 gene variants may be associated with obesity-related hypertension (Iwai et al. 1994, 2002, Telgmann et al. 2007, Tikhonoff et al. 2008). The mechanisms by which ACSM3 gene variants affect blood pressure remain to be elucidated.

Mitochondrial acyl-coenzyme A synthetase ACSM6 is proposed to be located in the mitochondrial matrix and possess the same medium-chain fatty acid:CoA ligase activity as ACSM3.
R-HSA-8933547 (Reactome) The ACP (acyl carrier protein) NDUFAB1 is the cofactor protein that covalently binds all fatty acyl intermediates via a phosphopantetheine linkage during the synthesis of fatty acids. Mitochondrial malonyl-CoA-acyl carrier protein transacylase (MCAT, MT) catalyses the transfer of a malonyl moiety from malonyl-CoA (Mal-CoA) to the free thiol group of the phosphopantetheine arm of NDUFAB1, suggesting a possible role in fatty acid biosynthesis in the mitochondrion (Zhang et al. 2003).
R-HSA-8952873 (Reactome) Mitochondrial trans-2-enoyl-CoA reductase (MECR aka NBRF1) is a dimeric oxidoreductase with a preference for short and medium chain trans fatty acyl-CoA substrates and may play a role in mitochondrial fatty acid synthesis (Chen et al. 2008). MECR is able to reduce trans acyl-CoA substrates of chain length C6 to C16 in an NADPH-dependent manner (Miinalainen et al. 2003). A representative reaction described here is the reduction of trans-dec-2-enoyl-CoA (tdec2-CoA) to decanoyl-CoA (DEC-CoA).
S-2MDPDA-CoAArrowR-HSA-5695980 (Reactome)
S-2MPDA-CoAR-HSA-5695980 (Reactome)
SUCC-CoAArrowR-HSA-71010 (Reactome)
Trifunctional ProteinArrowR-HSA-109339 (Reactome)
Trifunctional ProteinArrowR-HSA-109342 (Reactome)
Trifunctional Proteinmim-catalysisR-HSA-77271 (Reactome)
Trifunctional Proteinmim-catalysisR-HSA-77277 (Reactome)
Trifunctional Proteinmim-catalysisR-HSA-77283 (Reactome)
Trifunctional Proteinmim-catalysisR-HSA-77301 (Reactome)
Trifunctional Proteinmim-catalysisR-HSA-77303 (Reactome)
Trifunctional Proteinmim-catalysisR-HSA-77304 (Reactome)
Trifunctional Proteinmim-catalysisR-HSA-77309 (Reactome)
Trifunctional Proteinmim-catalysisR-HSA-77321 (Reactome)
Trifunctional Proteinmim-catalysisR-HSA-77329 (Reactome)
Trifunctional Proteinmim-catalysisR-HSA-77340 (Reactome)
acyl-CoAR-HSA-8874745 (Reactome)
adenosine 5'-monophosphateArrowR-HSA-5696004 (Reactome)
adenosine 5'-monophosphateArrowR-HSA-8875013 (Reactome)
cis,cis-3,6-Dodecadienoyl-CoAArrowR-HSA-109339 (Reactome)
cis,cis-3,6-Dodecadienoyl-CoAR-HSA-109338 (Reactome)
tdec2-CoAArrowR-HSA-109998 (Reactome)
tdec2-CoAArrowR-HSA-77345 (Reactome)
tdec2-CoAR-HSA-77344 (Reactome)
tdec2-CoAR-HSA-8952873 (Reactome)
trans,cis-Lauro-2,6-dienoyl-CoAArrowR-HSA-109338 (Reactome)
trans,cis-Lauro-2,6-dienoyl-CoAR-HSA-109342 (Reactome)
trans-Hex-2-enoyl-CoAArrowR-HSA-77327 (Reactome)
trans-Hex-2-enoyl-CoAR-HSA-77325 (Reactome)
trans-Hexadec-2-enoyl-CoAArrowR-HSA-77299 (Reactome)
trans-Hexadec-2-enoyl-CoAR-HSA-77301 (Reactome)
trans-Oct-2-enoyl-CoAArrowR-HSA-77338 (Reactome)
trans-Oct-2-enoyl-CoAR-HSA-77333 (Reactome)
trans-Tetradec-2-enoyl-CoAArrowR-HSA-77274 (Reactome)
trans-Tetradec-2-enoyl-CoAR-HSA-77277 (Reactome)

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