Glycerol metabolism including linked IMDs (Homo sapiens)

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Description

Glycerol metabolism, linked to inherited metabolic disorders (IMD).

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Ontology Terms

 

Bibliography

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  6. Timson DJ; ''Fructose 1,6-bisphosphatase: getting the message across.''; Biosci Rep, 2019 PubMed Europe PMC Scholia
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  12. Kashii T, Gomi T, Oya T, Ishii Y, Oda H, Maruyama M, Kobayashi M, Masuda T, Yamazaki M, Nagata T, Tsukada K, Nakajima A, Tatsu K, Mori H, Takusagawa F, Ogawa H, Pitot HC; ''Some biochemical and histochemical properties of human liver serine dehydratase.''; Int J Biochem Cell Biol, 2005 PubMed Europe PMC Scholia
  13. Rani RM, Syngkli S, Nongkhlaw J, Das B; ''Expression and characterisation of human glycerol kinase: the role of solubilising agents and molecular chaperones.''; Biosci Rep, 2023 PubMed Europe PMC Scholia
  14. Asipu A, Hayward BE, O'Reilly J, Bonthron DT; ''Properties of normal and mutant recombinant human ketohexokinases and implications for the pathogenesis of essential fructosuria.''; Diabetes, 2003 PubMed Europe PMC Scholia
  15. Sánchez-Gutiérrez JC, Benlloch T, Leal MA, Samper B, García-Ripoll I, Felíu JE; ''Molecular analysis of the aldolase B gene in patients with hereditary fructose intolerance from Spain.''; J Med Genet, 2002 PubMed Europe PMC Scholia
  16. Wexler ID, Kerr DS, Du Y, Kaung MM, Stephenson W, Lusk MM, Wappner RS, Higgins JJ; ''Molecular characterization of pyruvate carboxylase deficiency in two consanguineous families.''; Pediatr Res, 1998 PubMed Europe PMC Scholia
  17. Huang H, Tang S, Ji M, Tang Z, Shimada M, Liu X, Qi S, Locasale JW, Roeder RG, Zhao Y, Li X; ''p300-Mediated Lysine 2-Hydroxyisobutyrylation Regulates Glycolysis.''; Mol Cell, 2018 PubMed Europe PMC Scholia
  18. Althaher AR; ''An Overview of Hormone-Sensitive Lipase (HSL).''; ScientificWorldJournal, 2022 PubMed Europe PMC Scholia
  19. Hitosugi T, Zhou L, Fan J, Elf S, Zhang L, Xie J, Wang Y, Gu TL, Alečković M, LeRoy G, Kang Y, Kang HB, Seo JH, Shan C, Jin P, Gong W, Lonial S, Arellano ML, Khoury HJ, Chen GZ, Shin DM, Khuri FR, Boggon TJ, Kang S, He C, Chen J; ''Tyr26 phosphorylation of PGAM1 provides a metabolic advantage to tumours by stabilizing the active conformation.''; Nat Commun, 2013 PubMed Europe PMC Scholia
  20. Latorre-Muro P, Baeza J, Armstrong EA, Hurtado-Guerrero R, Corzana F, Wu LE, Sinclair DA, López-Buesa P, Carrodeguas JA, Denu JM; ''Dynamic Acetylation of Phosphoenolpyruvate Carboxykinase Toggles Enzyme Activity between Gluconeogenic and Anaplerotic Reactions.''; Mol Cell, 2018 PubMed Europe PMC Scholia
  21. Bollong MJ, Lee G, Coukos JS, Yun H, Zambaldo C, Chang JW, Chin EN, Ahmad I, Chatterjee AK, Lairson LL, Schultz PG, Moellering RE; ''A metabolite-derived protein modification integrates glycolysis with KEAP1-NRF2 signalling.''; Nature, 2018 PubMed Europe PMC Scholia
  22. Ferreras C, Hernández ED, Martínez-Costa OH, Aragón JJ; ''Subunit interactions and composition of the fructose 6-phosphate catalytic site and the fructose 2,6-bisphosphate allosteric site of mammalian phosphofructokinase.''; J Biol Chem, 2009 PubMed Europe PMC Scholia
  23. Sass JO, Fischer K, Wang R, Christensen E, Scholl-Bürgi S, Chang R, Kapelari K, Walter M; ''D-glyceric aciduria is caused by genetic deficiency of D-glycerate kinase (GLYCTK).''; Hum Mutat, 2010 PubMed Europe PMC Scholia
  24. Morais M, Dias F, Teixeira AL, Medeiros R; ''MicroRNAs and altered metabolism of clear cell renal cell carcinoma: Potential role as aerobic glycolysis biomarkers.''; Biochim Biophys Acta Gen Subj, 2017 PubMed Europe PMC Scholia
  25. Oh S, Mai XL, Kim J, de Guzman ACV, Lee JY, Park S; ''Glycerol 3-phosphate dehydrogenases (1 and 2) in cancer and other diseases.''; Exp Mol Med, 2024 PubMed Europe PMC Scholia
  26. O'connor T, Ireland LS, Harrison DJ, Hayes JD; ''Major differences exist in the function and tissue-specific expression of human aflatoxin B1 aldehyde reductase and the principal human aldo-keto reductase AKR1 family members.''; Biochem J, 1999 PubMed Europe PMC Scholia
  27. Menaya J, González-Manchón C, Parrilla R, Ayuso MS; ''Molecular cloning, sequencing and expression of a cDNA encoding a human liver NAD-dependent alpha-glycerol-3-phosphate dehydrogenase.''; Biochim Biophys Acta, 1995 PubMed Europe PMC Scholia
  28. Sass JO, Behringer S, Fernando M, Cesaroni E, Cursio I, Volpini A, Till C; ''d-Glycerate kinase deficiency in a neuropediatric patient.''; Brain Dev, 2020 PubMed Europe PMC Scholia
  29. TSUBOI KK, ESTRADA J, HUDSON PB; ''Enzymes of the human erythrocyte. IV. Phosphoglucose isomerase, purification and properties.''; J Biol Chem, 1958 PubMed Europe PMC Scholia
  30. Dionisi-Vici C, Shteyer E, Niceta M, Rizzo C, Pode-Shakked B, Chillemi G, Bruselles A, Semeraro M, Barel O, Eyal E, Kol N, Haberman Y, Lahad A, Diomedi-Camassei F, Marek-Yagel D, Rechavi G, Tartaglia M, Anikster Y; ''Expanding the molecular diversity and phenotypic spectrum of glycerol 3-phosphate dehydrogenase 1 deficiency.''; J Inherit Metab Dis, 2016 PubMed Europe PMC Scholia
  31. Katebi AR, Jernigan RL; ''The critical role of the loops of triosephosphate isomerase for its oligomerization, dynamics, and functionality.''; Protein Sci, 2014 PubMed Europe PMC Scholia
  32. Lebeck J; ''Metabolic impact of the glycerol channels AQP7 and AQP9 in adipose tissue and liver.''; J Mol Endocrinol, 2014 PubMed Europe PMC Scholia
  33. Cramer SD, Ferree PM, Lin K, Milliner DS, Holmes RP; ''The gene encoding hydroxypyruvate reductase (GRHPR) is mutated in patients with primary hyperoxaluria type II.''; Hum Mol Genet, 1999 PubMed Europe PMC Scholia
  34. Zhang L, Yao D, Xia Y, Zhou F, Zhang Q, Wang Q, Qin A, Zhao J, Li D, Li Y, Zhou L, Cao Y; ''The structural basis for glycerol permeation by human AQP7.''; Sci Bull (Beijing), 2021 PubMed Europe PMC Scholia

History

View all...
CompareRevisionActionTimeUserComment
140119view14:33, 30 July 2025Sander Lemmensvisual improvements
140118view14:28, 30 July 2025Sander LemmensVisual improvements and removed duplicate enzyme
140083view13:52, 24 July 2025Sander LemmensReverted to version '13:39, 24 July 2025' by Sander Lemmens
140082view13:45, 24 July 2025Sander LemmensReverted to version '16:21, 21 July 2025' by Sander Lemmens
140081view13:39, 24 July 2025DeSlTrying to fix issue with RHEA 10301 annotation
140080view13:37, 24 July 2025DeSlAliging items to the left bottom side vertically
140078view13:37, 22 July 2025Sander Lemmenscorrection reaction
140071view07:10, 22 July 2025Sander Lemmenssmall adjustments for visuals
140068view16:21, 21 July 2025Sander Lemmenscorrected error
140007view14:07, 17 July 2025Sander Lemmensadjustment of node
139983view09:03, 15 July 2025Sander Lemmensadded comment for reaction D-glyceralaldehyde to glycerate
139909view09:20, 10 July 2025DeSlOntology Term : 'D-glyceric aciduria' added !
139908view09:18, 10 July 2025DeSlOntology Term : 'glycerol kinase deficiency pathway' added !
139907view09:18, 10 July 2025DeSlOntology Term : 'glycerol kinase deficiency' added !
139899view06:54, 10 July 2025Sander Lemmensadded translocation to legend
139753view08:57, 3 July 2025Sander Lemmensremoved reaction from d-glyceraldehyde to r-glycerate
139714view10:55, 1 July 2025Sander Lemmenscrrections
139712view10:30, 1 July 2025Sander Lemmenscerrection fructose datanode
139709view07:46, 1 July 2025Sander LemmensAdded conversions to HSl
139696view17:11, 29 June 2025Sander Lemmenscorrections
139695view17:03, 29 June 2025Sander Lemmenscorrections
139694view16:56, 29 June 2025Sander Lemmenscorrections
139693view16:51, 29 June 2025Sander Lemmensadded datanodes,reaction and description
139692view15:46, 29 June 2025Sander Lemmensadded datanodes and reactions
139655view09:13, 27 June 2025Sander Lemmensedited layout and added datanodes and reactions
139654view05:56, 27 June 2025EgonwModified description
139639view13:23, 26 June 2025Sander Lemmensedited the layout, added datanodes and reactions
139634view09:25, 26 June 2025Sander Lemmenscrrected rhea reactions and added information on datanodes
139607view07:45, 23 June 2025Sander Lemmensadded information on data nodes
139477view12:41, 19 June 2025Sander LemmensCorrected G3P and GPD2, added information to data nodes and interactions
139470view03:44, 19 June 2025EgonwModified description
139469view03:44, 19 June 2025EgonwSmall typo
139468view03:43, 19 June 2025EgonwOntology Term : 'disease pathway' added !
139467view03:43, 19 June 2025EgonwOntology Term : 'classic metabolic pathway' added !
139438view09:16, 17 June 2025Sander Lemmenslinked diseases to interactions, added FFA
139416view13:32, 16 June 2025Sander Lemmensadjusted rhea reaction
139413view11:24, 16 June 2025Sander LemmensA few alignments
139412view11:16, 16 June 2025Sander LemmensNew pathway

External references

DataNodes

View all...
NameTypeDatabase referenceComment
(2R)-2-phosphoglycerateMetabolite58289 (ChEBI)
(2R)-3-phospho-glyceroyl phosphateMetabolite57604 (ChEBI)
(2R)-3-phosphoglycerateMetabolite58272 (ChEBI)
(2R)-3-phosphoglycerateMetaboliteCHEBI:58272 (ChEBI)
(R)-glycerateMetaboliteCHEBI:16659 (ChEBI)
AQP7ProteinO14520 (Uniprot-TrEMBL)
AQP9ProteinO43315 (Uniprot-TrEMBL)
ATGLProteinQ96AD5 (Uniprot-TrEMBL)
AldolaseProteinP04075 (Uniprot-TrEMBL)
D-glyceraldehydeMetaboliteCHEBI:17378 (ChEBI)
DHAPMetaboliteCHEBI:16108 (ChEBI)
DiacylglycerolMetaboliteCHEBI:88376 (ChEBI)
EnolaseProteinP06733 (Uniprot-TrEMBL)
FFAsMetaboliteCHEBI:28868 (ChEBI)
Fructose-1,6-bisphosphatase 1ProteinP09467 (Uniprot-TrEMBL)
GAPDHProteinP04406 (Uniprot-TrEMBL)
GKProteinP32189 (Uniprot-TrEMBL)
GLYCTKProteinQ8IVS8 (Uniprot-TrEMBL)
GPD1ProteinP21695 (Uniprot-TrEMBL)
GPD2ProteinP43304 (Uniprot-TrEMBL)
Glucose-6-phosphataseProteinP35575 (Uniprot-TrEMBL)
Glucose-6-phosphate isomeraseProteinP06744 (Uniprot-TrEMBL)
GlucoseMetaboliteCHEBI:17925 (ChEBI)
Glyceraldehyde-3-P MetaboliteCHEBI:59776 (ChEBI) Is D-glyceraldehyde 3-phosphate
Glycerol Metabolite17754 (ChEBI)
Glycerol-3-PMetaboliteCHEBI:57597 (ChEBI) Is sn-glycerol 3-phosphate
HSLProteinQ05469 (Uniprot-TrEMBL)
Hexokinase-1ProteinP19367 (Uniprot-TrEMBL)
L-serineMetaboliteCHEBI:17115 (ChEBI)
MGLLProteinQ99685 (Uniprot-TrEMBL)
MonoacylglycerolMetaboliteCHEBI:35759 (ChEBI)
Phosphoenolpyruvate carboxykinaseProteinP35558 (Uniprot-TrEMBL)
Phosphofructokinase 1 ProteinP17858 (Uniprot-TrEMBL)
Phosphoglycerate kinase 1ProteinP00558 (Uniprot-TrEMBL)
Phosphoglycerate mutase 1 ProteinP18669 (Uniprot-TrEMBL)
Phosphoglycerate mutase 1ProteinP18669 (Uniprot-TrEMBL)
Pyruvate carboxylaseProteinP11498 (Uniprot-TrEMBL)
Pyruvate kinaseProteinP30613 (Uniprot-TrEMBL)
TriacylglycerolMetaboliteCHEBI:88875 (ChEBI)
Triosephosphate isomeraseProteinP60174 (Uniprot-TrEMBL)
fructoseMetaboliteCHEBI:28757 (ChEBI)
oxaloacetateMetaboliteCHEBI:16452 (ChEBI)
phosphoenolpyruvateMetabolite58702 (ChEBI)
pyruvateMetaboliteCHEBI:15361 (ChEBI)
α-D-glucose 6-phosphateMetaboliteCHEBI:58225 (ChEBI)
β-D-fructose 1,6-bisphosphateMetaboliteCHEBI:32966 (ChEBI)
β-D-fructose 6-phosphateMetaboliteCHEBI:57634 (ChEBI)

Annotated Interactions

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SourceTargetTypeDatabase referenceComment
(2R)-2-phosphoglycerate(2R)-3-phosphoglyceratemim-conversion15904 (Rhea)
(2R)-3-phospho-glyceroyl phosphateGlyceraldehyde-3-P mim-conversion10302 (Rhea)
(2R)-3-phosphoglycerate(2R)-2-phosphoglyceratemim-conversion15903 (Rhea)
(2R)-3-phosphoglycerate(2R)-3-phospho-glyceroyl phosphatemim-conversion14802 (Rhea)
(R)-glycerate(2R)-3-phosphoglyceratemim-conversion23517 (Rhea)
DHAPGlycerol-3-Pmim-conversion11094 (Rhea)
DiacylglycerolMonoacylglycerolmim-conversion32732 (Rhea)
Glucoseα-D-glucose 6-phosphatemim-conversion36496 (Rhea)
Glyceraldehyde-3-P DHAPmim-conversion18588 (Rhea)
Glyceraldehyde-3-P β-D-fructose 1,6-bisphosphatemim-conversion14732 (Rhea)
Glycerol Glycerol Arrow29678 (Rhea)
Glycerol Glycerol-3-Pmim-conversion21645 (Rhea)
Glycerol-3-PDHAPmim-conversion18978 (Rhea)
MonoacylglycerolGlycerol mim-conversion15246 (Rhea)
TriacylglycerolDiacylglycerolmim-conversion12045 (Rhea)
oxaloacetatephosphoenolpyruvatemim-conversion10389 (Rhea)
phosphoenolpyruvate(2R)-2-phosphoglyceratemim-conversion10167 (Rhea)
phosphoenolpyruvatepyruvatemim-conversion18159 (Rhea)
pyruvateoxaloacetatemim-conversion20845 (Rhea)
α-D-glucose 6-phosphateGlucosemim-conversion44905 (Rhea)
α-D-glucose 6-phosphateβ-D-fructose 6-phosphatemim-conversion11819 (Rhea)
β-D-fructose 1,6-bisphosphateGlyceraldehyde-3-P mim-conversion14732 (Rhea)
β-D-fructose 1,6-bisphosphateβ-D-fructose 6-phosphatemim-conversion11065 (Rhea)
β-D-fructose 6-phosphateβ-D-fructose 1,6-bisphosphatemim-conversion16110 (Rhea)
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