Biotin metabolism, including IMDs (Homo sapiens)
From WikiPathways
Description
This pathway was inspired by Chapter 14 (edition 4) of the book of Blau (ISBN 3642403360 (978-3642403361)).
Quality Tags
Ontology Terms
Bibliography
- 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
- Hymes J, Wolf B; ''Biotinidase and its roles in biotin metabolism.''; Clin Chim Acta, 1996 PubMed Europe PMC Scholia
- Said HM; ''Biotin: biochemical, physiological and clinical aspects.''; Subcell Biochem, 2012 PubMed Europe PMC Scholia
History
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External references
DataNodes
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Name | Type | Database reference | Comment |
---|---|---|---|
3-methylcortonyl-COA | Metabolite | CHEBI:57344 (ChEBI) | aka 3-methylbut-2-enoyl-CoA |
3-methylglutaconyl-CoA | Metabolite | CHEBI:57346 (ChEBI) | aka trans-3-methylglutaconyl-CoA |
ACC1 (cytosol) | Protein | Q13085 (Uniprot-TrEMBL) | aka acetyl-CoA carboxylase |
ACC2 (mitochondrial) | Protein | O00763 (Uniprot-TrEMBL) | aka acetyl-CoA carboxylase |
Acetyl-CoA | Metabolite | CHEBI:57288 (ChEBI) | |
Anaplerosis | Pathway | ||
Apo-ACC | Metabolite | CHEBI:29969 (ChEBI) | aka apo-[methylmalonyl-CoA:pyruvate carboxytransferase] |
Apo-MCC | Metabolite | CHEBI:29969 (ChEBI) | aka apo-[3-methylcrotonoyl-CoA:carbon-dioxide ligase (ADP-forming)] |
Apo-PCC | Metabolite | CHEBI:29969 (ChEBI) | aka apo-[propionyl-CoA:carbon-dioxide ligase (ADP-forming)] |
Apo-PC | Metabolite | CHEBI:29969 (ChEBI) | aka pyruvate carboxylase |
Biocytin | Metabolite | Q864247 (Wikidata) | biotin covalantly bound to lysine |
Biotin (free pool) | Metabolite | CHEBI:15956 (ChEBI) | |
Biotin | Metabolite | CHEBI:15956 (ChEBI) | |
Biotinidase (BTD) | Protein | P43251 (Uniprot-TrEMBL) | |
Dietary and other protein-bound biotin | Metabolite | CHEBI:16615 (ChEBI) | |
Fatty Acid Synthesis | Pathway | ||
Gluconeogenisis | Pathway | ||
HLCS | Protein | P50747 (Uniprot-TrEMBL) | aka Holocarboxylase Synthetase |
Leucine Catabolism | Pathway | ||
Lysine | Metabolite | CHEBI:25094 (ChEBI) | |
MCCC1 | Protein | Q96RQ3 (Uniprot-TrEMBL) | |
MCCC2 | Protein | Q9HCC0 (Uniprot-TrEMBL) | |
Malonyl-CoA | Metabolite | CHEBI:57384 (ChEBI) | |
PCCA | Protein | P05165 (Uniprot-TrEMBL) | |
PCCB | Protein | P05166 (Uniprot-TrEMBL) | |
Propionate metabolism | Pathway | ||
Proteolysis | Protein | ||
free biotin | Metabolite | CHEBI:15956 (ChEBI) | |
holo-ACC | Metabolite | CHEBI:83144 (ChEBI) | aka holo-[methylmalonyl-CoA:pyruvate carboxytransferase] |
holo-MCC | Metabolite | CHEBI:83144 (ChEBI) | aka holo-[3-methylcrotonoyl-CoA:carbon-dioxide ligase (ADP-forming)] |
holo-PCC | Metabolite | CHEBI:83144 (ChEBI) | aka holo-[propionyl-CoA:carbon-dioxide ligase (ADP-forming)] |
holo-PC | Metabolite | CHEBI:83144 (ChEBI) | aka pyruvate carboxylase |
leucine | Metabolite | CHEBI:25017 (ChEBI) |
Annotated Interactions
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Source | Target | Type | Database reference | Comment |
---|---|---|---|---|
3-methylcortonyl-COA | 3-methylglutaconyl-CoA | mim-conversion | 13590 (Rhea) | |
Acetyl-CoA | Malonyl-CoA | mim-conversion | 11309 (Rhea) | |
Apo-ACC | mim-conversion | 23669 (Rhea) | ||
Apo-MCC | mim-conversion | 24377 (Rhea) | ||
Apo-PCC | mim-conversion | 11205 (Rhea) | ||
Apo-PC | mim-conversion | 11757 (Rhea) | ||
Biocytin | Biotin (free pool) | mim-conversion | 13082 (Rhea) | |
Biotin (free pool) | mim-conversion | 11205 (Rhea) | ||
Biotin (free pool) | mim-conversion | 11757 (Rhea) | ||
Biotin (free pool) | mim-conversion | 23669 (Rhea) | ||
Biotin (free pool) | mim-conversion | 24377 (Rhea) | ||
Lysine | mim-conversion | 13082 (Rhea) |