Cholesterol metabolism with Bloch and Kandutsch-Russell pathways (Homo sapiens)

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ArcPathVisio Brace Ellipse EndoplasmicReticulum GolgiApparatus HexagonPathVisio MimDegradation Mitochondria Octagon PentagonPathVisio Rectangle RoundedRectangle SarcoplasmicReticulum TriangleEquilateralEast TrianglePathVisio none LXRalpha/betaSterol effluxothers9Z-palmitoleic acidOleic acidSREBF17alpha-hydroxycholesterolBloch PathwayAcetyl-CoACH25HHMGCRDHCR7DHCR24SOAT1SOAT2FASNACSL1ACSL3ACSL4ELOVL2ELOVL3ELOVL4ELOVL5FADS1FADS2SCDScd2Acot1ACOT2NR1H3NR1H2ABCA1ABCG1CE(16:1)Cholesteryl esters (CE)CE(18:1)Acetoacetyl-CoAHMG-CoAMevalonic acidMevalonate-5-PMevalonate-5-PPIsopentenyl-PPDimethylallyl-PPGeranyl-PPFarnesyl-PPSqualeneSqualene-2,3-epoxideDiepoxy-squaleneLanosterol24,25-epoxycholesterol24,25-dihydrolanosterol32-hydroxylanosterol4,4-dimethylcholesta-5,8,24-trienol4,4-dimethylcholest-8-enol14-demethyl-lanosterol4-alpha-methylcholest-8-enolZymosterol4-alpha-methyl-cholest-8-enoneZymostenolLathosterol7-dehdrocholesterolCholestadienol7-dehydodesmosterolDesmosterolCholesterolSC5DEBPNSDHLMSMO1HSD17B7CYP51A1TM7SF2CYP51A1DHCR247-oxocholesterolCholestenone4beta-hydroxycholesterol24S-hydroxycholesterol25-hydroxycholesterol27-hydroxycholesterolCYP46A1CYP27A1Acyl-CoAACAT2HMGCS1HMGCS2MVKPMVKMVDSQLEIDI1Idi2FDFT1GGPS1FDPSLSSKandutsch-Russell PathwayPreSqualeneDHCR7DHCR24MYLIPLDLR degradationSREBF2Sterol biosynthesisFatty acid biosynthesis4-methyl zymostenol4-methyl zymostenone4-methyl-4-carboxyzymostenonedihydro-FF-MASdihydro-T-MASCYP51A1TM7SF2LBRMSMO1NSDHL4-methyl zymosterol4-methyl zymosterone4-methyl-4-carboxyzymosteroneFF-MAST-MASTM7SF2LBRMSMO1NSDHLHSD17B7MSMO1NSDHLHSD17B7CYP51A1HSD17B7Name: Cholesterol metabolism with Bloch and Kandutsch-Russell pathwaysOrganism: Homo sapiens


Description

This pathway is inspired by the [Lipidmaps>Sterol lipids expended pathway display](https://lipidmaps.org/pathway/pathways_maps) and extended with Scheme 1 from [Acimovic et al 2013](https://www.ncbi.nlm.nih.gov/pubmed/23558541). Literature suggests that cholesterol synthesis preferentially starts with the Bloch pathway, however there is a shift to the Kandutsch-Russell part via lathosterol ([Bae et al, 1997](https://www.ncbi.nlm.nih.gov/pubmed/9291139)). Dashed lines indicate that multiple steps are involved to create the final product. Several regulatory effects concerning the metabolites of cholesterol have been indicated as well. The content from the Bloch and Kandutsch-Russel pathways have been checked against literature, and differences compared to the original LipidMaps pathway have been coloured turquoise.

Comments

HomologyMapper 
This pathway was inferred from Mus musculus pathway [WP4346_106325.gpml] with a 94.0% conversion rate.

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Quality Tags

Image:Wplogo_31.pngCommunity: Lipids and LIPID MAPS
Image:Curated.pngApproved version
Image:MissingXref.pngAnnotate nodes
Homology Converted

Ontology Terms

Pathway Ontology : protein degradation pathway
 

Bibliography

View all...
  1. Dennis EA, Deems RA, Harkewicz R, Quehenberger O, Brown HA, Milne SB, Myers DS, Glass CK, Hardiman G, Reichart D, Merrill AH Jr, Sullards MC, Wang E, Murphy RC, Raetz CR, Garrett TA, Guan Z, Ryan AC, Russell DW, McDonald JG, Thompson BM, Shaw WA, Sud M, Zhao Y, Gupta S, Maurya MR, Fahy E, Subramaniam S; ''A mouse macrophage lipidome.''; J Biol Chem, 2010 PubMed Europe PMC Scholia
  2. Ačimovič J, Rozman D; ''Steroidal triterpenes of cholesterol synthesis.''; Molecules, 2013 PubMed Europe PMC Scholia
  3. Bae SH, Paik YK; ''Cholesterol biosynthesis from lanosterol: development of a novel assay method and characterization of rat liver microsomal lanosterol delta 24-reductase.''; Biochem J, 1997 PubMed Europe PMC Scholia
  4. Ačimovič J, Rozman D; ''Steroidal triterpenes of cholesterol synthesis.''; Molecules, 2013 PubMed Europe PMC Scholia
  5. Ačimovič J, Rozman D; ''Steroidal triterpenes of cholesterol synthesis.''; Molecules, 2013 PubMed Europe PMC Scholia
  6. Bogan RL, Debarber AE, Hennebold JD; ''Liver x receptor modulation of gene expression leading to proluteolytic effects in primate luteal cells.''; Biol Reprod, 2012 PubMed Europe PMC Scholia

History

View all...
CompareRevisionActionTimeUserComment
139400
Lipids
Approved
view08:48, 14 June 2025EgonwRemoved duplicate BioPax fields
139394view19:18, 13 June 2025KhanspersModified description
139361view23:53, 5 June 2025KhanspersModified description
137920view17:46, 10 March 2025KhanspersModified description
125822view18:19, 12 March 2023EgonwThe linked pathway is for mouse without an human equivalent
124755view08:57, 8 December 2022DeSlUpdated link to LIPID MAPS PWs.
119764view00:12, 19 August 2021KhanspersUpdated annotation for ACAT2 which was misannotated before
117681view12:05, 22 May 2021EweitzModified title
107595view15:13, 23 October 2019DeSlOntology Term : 'lipid signaling pathway' added !
107594view15:12, 23 October 2019DeSlOntology Term : 'cholesterol metabolic pathway' added !
107593view15:12, 23 October 2019DeSlOntology Term : 'PW:0000002' removed !
107592view15:12, 23 October 2019DeSlOntology Term : 'cholesterol biosynthetic pathway' added !
107587view15:09, 23 October 2019DeSlNew pathway

External references

DataNodes

View all...
Name  ↓Type  ↓Database reference  ↓Comment  ↓
14-demethyl-lanosterolMetaboliteLMST01010176 (LIPID MAPS)
24,25-dihydrolanosterolMetaboliteLMST01010087 (LIPID MAPS)
24,25-epoxycholesterolMetaboliteLMST01010012 (LIPID MAPS)
24S-hydroxycholesterolMetaboliteLMST01010019 (LIPID MAPS)
25-hydroxycholesterolMetaboliteLMST01010018 (LIPID MAPS)
27-hydroxycholesterolMetaboliteLMST01010057 (LIPID MAPS)
32-hydroxylanosterolMetaboliteLMST01010124 (LIPID MAPS)
4,4-dimethylcholest-8-enolMetaboliteLMST01010225 (LIPID MAPS)
4,4-dimethylcholesta- 5,8,24-trienolMetaboliteLMST01010149 (LIPID MAPS)
4-alpha-methyl-cholest-8-enoneMetaboliteLMST01010236 (LIPID MAPS)
4-alpha-methylcholest-8-enolMetaboliteLMST01010197 (LIPID MAPS)
4-methyl zymostenolMetabolite22212495 (PubChem-compound)
4-methyl zymostenoneMetaboliteC12CC[C@@]3([H])C(C)[C](=O)CC[C@]3(C)C=1CC[C@]1(C)[C@@]([H])([C@@](C)([H])CC/CC(\C)/C)CC[C]21H
4-methyl zymosterolMetaboliteC12CC[C@@]3([H])C(C)[C@@H](O)CC[C@]3(C)C=1CC[C@]1(C)[C@@]([H])([C@@](C)([H])CC/C=C(\C)/C)CC[C]21H
4-methyl zymosteroneMetaboliteC12CC[C@@]3([H])C(C)[C](=O)CC[C@]3(C)C=1CC[C@]1(C)[C@@]([H])([C@@](C)([H])CC/C=C(\C)/C)CC[C]21H
4-methyl-4-carboxy zymostenoneMetaboliteC12CC[C@@]3([H])C(C)(COOH)[C@@H](O)CC[C@]3(C)C=1CC[C@]1(C)[C@@]([H])([C@@](C)([H])CC/CC(\C)/C)CC[C]21H
4-methyl-4-carboxy zymosteroneMetaboliteC12CC[C@@]3([H])C(C)(COOH)[C@@H](O)CC[C@]3(C)C=1CC[C@]1(C)[C@@]([H])([C@@](C)([H])CC/C=C(\C)/C)CC[C]21H
4beta-hydroxycholesterolMetaboliteLMST01010014 (LIPID MAPS)
7-dehdrocholesterolMetaboliteLMST01010069 (LIPID MAPS)
7-dehydodesmosterolMetaboliteLMST01010121 (LIPID MAPS)
7-oxocholesterolMetaboliteLMST01010049 (LIPID MAPS)
7alpha-hydroxycholesterolMetaboliteLMST01010013 (LIPID MAPS)
9Z-palmitoleic acidMetabolite445638 (PubChem-compound) Assuming this is omega 7-type
ABCA1GeneProductENSG00000165029 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = En:ENSMUSG00000015243
ABCG1GeneProductENSG00000160179 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = En:ENSMUSG00000024030
ACAT2GeneProductENSG00000120437 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:171210
ACOT2GeneProductENSG00000119673 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = En:ENSMUSG00000021226
ACSL1GeneProductENSG00000151726 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = En:ENSMUSG00000018796
ACSL3GeneProductENSG00000123983 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = En:ENSMUSG00000032883
ACSL4GeneProductENSG00000068366 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = En:ENSMUSG00000031278
Acetoacetyl-CoAMetaboliteLMFA07050030 (LIPID MAPS)
Acetyl-CoAMetaboliteLMFA07050281 (LIPID MAPS)
Acot1GeneProductNo homologues found for original id En:ENSMUSG00000072949
Acyl-CoAMetaboliteLMFA07050000 (LIPID MAPS)
Bloch PathwayPathway
CE(16:1)MetaboliteQ412366 (Wikidata) Assuming this is omega-7 type
CE(18:1)MetaboliteQ27116670 (Wikidata) Assuming this is omega-7 type
CH25HGeneProductENSG00000138135 (Ensembl)
  • Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:12642
CYP27A1GeneProductENSG00000135929 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:104086
CYP46A1GeneProductENSG00000036530 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:13116
CYP51A1ProteinENSG00000001630 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = S:Q8K0C4
CholestadienolMetaboliteLMST01010206 (LIPID MAPS) aka 24-dehydrolathosterol
CholestenoneMetaboliteLMST01010015 (LIPID MAPS)
CholesterolMetaboliteLMST01010001 (LIPID MAPS)
Cholesteryl esters (CE)MetaboliteCHEBI:17002 (ChEBI)
DHCR24GeneProductENSG00000116133 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:74754
DHCR7GeneProductENSG00000172893 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:13360
DesmosterolMetaboliteLMST01010016 (LIPID MAPS)
Diepoxy-squaleneMetaboliteLMPR0106010038 (LIPID MAPS)
Dimethylallyl-PPMetaboliteLMPR01010001 (LIPID MAPS)
EBPGeneProductENSG00000147155 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:13595
ELOVL2ProteinENSG00000197977 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = En:ENSMUSG00000021364
ELOVL3ProteinENSG00000119915 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = En:ENSMUSG00000038754
ELOVL4ProteinENSG00000118402 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = En:ENSMUSG00000032262
ELOVL5ProteinENSG00000012660 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = En:ENSMUSG00000032349
FADS1ProteinENSG00000149485 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = En:ENSMUSG00000010663
FADS2ProteinENSG00000134824 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = En:ENSMUSG00000024665
FASNGeneProductENSG00000169710 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = En:ENSMUSG00000025153
FDFT1GeneProductENSG00000079459 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:14137
FDPSGeneProductENSG00000160752 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:110196
FF-MASMetabolite64284-64-6 (CAS)
  • aka 14-demethyl-14-dehydrolanosterol
  • C12CC[C@@]3([H])C(C)(C)[C@@H](O)CC[C@]3(C)C=1CC[C@]1(C)[C@@]([H])([C@@](C)([H])CC/C=C(\C)/C)CC=[C@@]21C SMILES
Farnesyl-PPMetaboliteLMPR0103010002 (LIPID MAPS)
Fatty acid biosynthesisPathway
GGPS1GeneProductENSG00000152904 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:14593
Geranyl-PPMetaboliteLMPR0102010001 (LIPID MAPS)
HMG-CoAMetaboliteLMFA07050116 (LIPID MAPS)
HMGCRGeneProductENSG00000113161 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:15357
HMGCS1GeneProductENSG00000112972 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:208715
HMGCS2GeneProductENSG00000134240 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:15360
HSD17B7ProteinENSG00000132196 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:15490
IDI1GeneProductENSG00000067064 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:319554
Idi2GeneProductNo homologues found for original id L:320581
Isopentenyl-PPMetaboliteLMPR01010008 (LIPID MAPS)
Kandutsch-

Russell

Pathway		Pathway
LBRProteinENSG00000143815 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = S:Q3U9G9
LSSGeneProductENSG00000160285 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:16987
LanosterolMetaboliteLMST01010017 (LIPID MAPS)
LathosterolMetaboliteLMST01010089 (LIPID MAPS)
MSMO1ProteinENSG00000052802 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = S:Q9CRA4
MVDGeneProductENSG00000167508 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:192156
MVKGeneProductENSG00000110921 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:17855
MYLIPGeneProductENSG00000007944 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = En:ENSMUSG00000038175
Mevalonate-5-PMetaboliteLMFA01050415 (LIPID MAPS)
Mevalonate-5-PPMetaboliteLMFA01050416 (LIPID MAPS)
Mevalonic acidMetaboliteLMFA01050352 (LIPID MAPS)
NR1H2GeneProductENSG00000131408 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = En:ENSMUSG00000060601
NR1H3GeneProductENSG00000025434 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = En:ENSMUSG00000002108
NSDHLProteinENSG00000147383 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = S:Q9R1J0
Oleic acidMetaboliteQ207688 (Wikidata) Aka C18:1 (omega 9)
PMVKGeneProductENSG00000163344 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:68603
PreSqualeneMetaboliteQ27088444 (Wikidata) Annotated while assuming this compound is actually presqualene-diphosphate
SC5DGeneProductENSG00000109929 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:235293
SCDProteinENSG00000099194 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = En:ENSMUSG00000037071
SOAT1GeneProductENSG00000057252 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:20652
SOAT2GeneProductENSG00000167780 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:223920
SQLEGeneProductENSG00000104549 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:20775
SREBF1GeneProductENSG00000072310 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = En:ENSMUSG00000020538
SREBF2GeneProductENSG00000198911 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = En:ENSMUSG00000022463
Scd2ProteinNo homologues found for original id S:P13011
Squalene-2,3-epoxideMetaboliteLMPR0106010010 (LIPID MAPS)
SqualeneMetaboliteLMPR0106010002 (LIPID MAPS)
T-MASMetaboliteC12CC[C@@]3([H])C(C)(C)[C@@H](O)CC[C@]3(C)C=1CC[C@]1(C)[C@@]([H])([C@@](C)([H])CC/C=C(\C)/C)CC[C]21H SMILES
TM7SF2ProteinENSG00000149809 (Ensembl) Homology Mapping from Mus musculus to Homo sapiens: Original ID = L:73166
ZymostenolMetaboliteLMST01010096 (LIPID MAPS)
ZymosterolMetaboliteLMST01010066 (LIPID MAPS)
dihydro-FF-MASMetaboliteC12CC[C@@]3([H])C(C)(C)[C@@H](O)CC[C@]3(C)C=1CC[C@]1(C)[C@@]([H])([C@@](C)([H])CC/CC(\C)/C)CC=[C@@]21C
dihydro-T-MASMetaboliteC12CC[C@@]3([H])C(C)(C)[C@@H](O)CC[C@]3(C)C=1CC[C@]1(C)[C@@]([H])([C@@](C)([H])CC/CC(\C)/C)CC[C]21H

Annotated Interactions

No annotated interactions
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