Biotin metabolism, including IMDs (Homo sapiens)

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mitochondriaACCs:MCCC13-methylglutaconyl-CoA3-methylcortonyl-COAFatty Acid SynthesisApo-ACCBiotinidase deficiency (BTD)Holocarboxylases(ACC, MCC, PCC, PC)Dietary and otherprotein-bound biotinProteolysisBiocytinLysineBiotinidase (BTD)free biotinAcetyl-CoABiotin (free pool)Apocarboxylases(ACC, MCC, PCC, PC)HLCSHolocar-boxylase synthetase deficiency(HCSD)Malonyl-CoAACC1(cytosol)ACC2(mitochondrial)LeucineCatabolismPropionatemetabolismGluconeogenisisAnaplerosisholo-ACCApo-PCCholo-PCCApo-MCCholo-MCCApo-PCholo-PCMCCs:MCCC2Biotin


Description

ch. 14 Blau book

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Quality Tags

Ontology Terms

 

Bibliography

  1. Nenad Blau, Carlo Dionisi Vici, K Michael Gibson, Marinus Duran; ''Physician's Guide to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases''; Springer, 2014
  2. Hymes J, Wolf B; ''Biotinidase and its roles in biotin metabolism.''; Clin Chim Acta, 1996 PubMed Europe PMC Scholia
  3. Said HM; ''Biotin: biochemical, physiological and clinical aspects.''; Subcell Biochem, 2012 PubMed Europe PMC Scholia

History

View all...
CompareRevisionActionTimeUserComment
140994view14:44, 31 October 2025EweitzStandardize case
140993view14:41, 31 October 2025EweitzEconomize layout
122564view09:46, 16 April 2022EgonwModified title
119099view09:43, 17 June 2021FinterlyAdded ISBN for book citation
117665view11:57, 22 May 2021EweitzModified title
115139view15:40, 28 January 2021EgonwMade four pathways clickable
114213view11:32, 25 December 2020DeSlUpdate description.
114212view11:32, 25 December 2020DeSlUpdated description.
114211view11:22, 25 December 2020DeSlAdded another lit. ref
114210view11:16, 25 December 2020DeSlAdded lit. ref for biotinidase
114209view11:14, 25 December 2020DeSlAdded last diseases.
114208view11:13, 25 December 2020DeSlAdded more diseases
114207view11:07, 25 December 2020DeSlLayout changes
114206view11:06, 25 December 2020DeSlCompeted last step
114205view11:00, 25 December 2020DeSlAnnotated PW nodes
114204view10:53, 25 December 2020DeSlAdded interactions for propionate
114203view10:27, 25 December 2020DeSlAdded general lit. ref.
114202view10:26, 25 December 2020DeSlConnected unconnected line
114201view10:25, 25 December 2020DeSlOntology Term : '3-methylcrotonyl CoA carboxylase 2 deficiency pathway' added !
114200view10:25, 25 December 2020DeSlOntology Term : '3-methylcrotonyl CoA carboxylase 1 deficiency pathway' added !
114199view10:24, 25 December 2020DeSlOntology Term : 'pyruvate carboxylase deficiency pathway' added !
114198view10:24, 25 December 2020DeSlOntology Term : 'pyruvate carboxylase deficiency disease' added !
114197view10:22, 25 December 2020DeSlOntology Term : '3-Methylcrotonyl-CoA carboxylase deficiency' added !
114196view10:22, 25 December 2020DeSlOntology Term : '3-Methylcrotonyl-CoA carboxylase 2 deficiency' added !
114195view10:21, 25 December 2020DeSlOntology Term : '3-Methylcrotonyl-CoA carboxylase 1 deficiency' added !
114194view10:21, 25 December 2020DeSlOntology Term : 'holocarboxylase synthetase deficiency pathway' added !
114193view10:21, 25 December 2020DeSlOntology Term : 'holocarboxylase synthetase deficiency' added !
114192view10:21, 25 December 2020DeSlOntology Term : 'biotin metabolic pathway' added !
114191view10:20, 25 December 2020DeSlOntology Term : 'biotinidase deficiency pathway' added !
114190view10:20, 25 December 2020DeSlOntology Term : 'biotinidase deficiency' added !
114189view10:20, 25 December 2020DeSlUpdated description
114188view16:25, 24 December 2020DeSlsmall updates
114187view16:13, 24 December 2020DeSlAdded interactions related to leucine metabolism
114186view15:45, 24 December 2020DeSlfinished proteolysis interactions
114185view15:20, 24 December 2020DeSlAdded first interactions on apo to holo caroxylases
114184view14:41, 24 December 2020DeSlfixed typo in title
114183view14:39, 24 December 2020DeSlNew pathway

External references

DataNodes

View all...
NameTypeDatabase referenceComment
3-methylcortonyl-COAMetaboliteCHEBI:57344 (ChEBI) aka 3-methylbut-2-enoyl-CoA
3-methylglutaconyl-CoAMetaboliteCHEBI:57346 (ChEBI) aka trans-3-methylglutaconyl-CoA
ACC1 (cytosol)ProteinQ13085 (Uniprot-TrEMBL) aka acetyl-CoA carboxylase
ACC2 (mitochondrial)ProteinO00763 (Uniprot-TrEMBL) aka acetyl-CoA carboxylase
Acetyl-CoAMetaboliteCHEBI:57288 (ChEBI)
AnaplerosisPathway
Apo-ACCMetaboliteCHEBI:29969 (ChEBI) aka apo-[methylmalonyl-CoA:pyruvate carboxytransferase]
Apo-MCCMetaboliteCHEBI:29969 (ChEBI) aka apo-[3-methylcrotonoyl-CoA:carbon-dioxide ligase (ADP-forming)]
Apo-PCCMetaboliteCHEBI:29969 (ChEBI) aka apo-[propionyl-CoA:carbon-dioxide ligase (ADP-forming)]
Apo-PCMetaboliteCHEBI:29969 (ChEBI) aka pyruvate carboxylase
BiocytinMetaboliteQ864247 (Wikidata) biotin covalantly bound to lysine
Biotin (free pool)MetaboliteCHEBI:15956 (ChEBI)
Biotin MetaboliteCHEBI:15956 (ChEBI)
Biotinidase (BTD)ProteinP43251 (Uniprot-TrEMBL)
Dietary and other protein-bound biotinMetaboliteCHEBI:16615 (ChEBI)
Fatty Acid SynthesisPathway
GluconeogenisisPathway
HLCSProteinP50747 (Uniprot-TrEMBL) aka Holocarboxylase Synthetase
Leucine CatabolismPathway
LysineMetaboliteCHEBI:25094 (ChEBI)
MCCC1ProteinQ96RQ3 (Uniprot-TrEMBL)
MCCC2ProteinQ9HCC0 (Uniprot-TrEMBL)
Malonyl-CoAMetaboliteCHEBI:57384 (ChEBI)
Propionate metabolismPathway
ProteolysisProtein
free biotinMetaboliteCHEBI:15956 (ChEBI)
holo-ACCMetaboliteCHEBI:83144 (ChEBI) aka holo-[methylmalonyl-CoA:pyruvate carboxytransferase]
holo-MCCMetaboliteCHEBI:83144 (ChEBI) aka holo-[3-methylcrotonoyl-CoA:carbon-dioxide ligase (ADP-forming)]
holo-PCCMetaboliteCHEBI:83144 (ChEBI) aka holo-[propionyl-CoA:carbon-dioxide ligase (ADP-forming)]
holo-PCMetaboliteCHEBI:83144 (ChEBI) aka pyruvate carboxylase

Annotated Interactions

View all...
SourceTargetTypeDatabase referenceComment
3-methylcortonyl-COA3-methylglutaconyl-CoAmim-conversion13590 (Rhea)
Acetyl-CoAMalonyl-CoAmim-conversion11309 (Rhea)
Apo-ACCmim-conversion23669 (Rhea)
Apo-MCCmim-conversion24377 (Rhea)
Apo-PCCmim-conversion11205 (Rhea)
Apo-PCmim-conversion11757 (Rhea)
BiocytinBiotin (free pool)mim-conversion13082 (Rhea)
Biotin (free pool)mim-conversion11205 (Rhea)
Biotin (free pool)mim-conversion11757 (Rhea)
Biotin (free pool)mim-conversion23669 (Rhea)
Biotin (free pool)mim-conversion24377 (Rhea)
Lysinemim-conversion13082 (Rhea)
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