Isovalerylglucuronide (BioCAD00000020064)

blood urine kidney liver

Metabolite Card

Formula: C11H18O8 (278.1002)
SMILES: CC(C)CC(=O)O[C@@H]1O[C@@H]([C@@H](O)[C@H](O)[C@H]1O)C(O)=O

Synonyms [en]

Isovalerylglucuronide; Isovaleryl-beta-D-glucuronide; 9XP74KZ672; (2S,3S,4S,5R,6S)-3,4,5-trihydroxy-6-[(3-methylbutanoyl)oxy]oxane-2-carboxylic acid; (2S,3S,4S,5R,6S)-3,4,5-trihydroxy-6-(3-methylbutanoyloxy)oxane-2-carboxylic acid; isovaleryglucuronide

Reviewed

Last reviewed on 2024-06-28.

Cite this Page

Isovalerylglucuronide. 数据之源，洞见之始. SMRUCC genomics institute, a synthetic life researcher from China. https://biocad_registry.innovation.ac.cn/s/(-)-arctiin (retrieved 2026-01-03) (CAD Registry RN: BioCAD00000020064). Licensed under the Attribution-Noncommercial 4.0 International License (CC BY-NC 4.0).

Note

Isovaleryl glucuronide has been identified in the urine of patients with isovaleric acidemia made possible by using gas chromatograph-mass spectrometry (GC-MS). Isovaleryl glucuronide is more likely to be excreted when the amount of urinary 3-hydroxyisovaleric acid excretion is high. Isovaleryl glucuronide is more likely to be excreted when the amount of urinary 3-hydroxyisovaleric acid excretion is high. (PMID: 6547525). Isovaleric acidemia is caused by mutation in the isovaleryl CoA dehydrogenase gene and is closely related to maple syrup urine disease. It presents in two forms: the acute neonatal form, leading to massive metabolic acidosis from the first days of life and rapid death and a chronic form in which periodic attacks of severe ketoacidosis occur with asymptomatic intervening periods. (OMIM 243500).

Cytoplasm
Entity Information

DBLinks

Other DBLinks
  • CAS Registry Number: 88070-93-3
  • PubChem: 137383
  • ChEBI: ChEBI:174651
  • HMDB: HMDB0002091
  • HMDB: HMDB02091
  • KEGG: C03033
  • NCBI MeSH: isovalerylglucuronide
  • RefMet: RM0200812
  • Metlin: METLIN_6482
  • Coconut NaturalProduct: CNP0200932.1

Class / Ontology

Metabolic Network
ID EC Number Name
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Organism Source

Taxonomy Source

Pathway Synthetic

pathway id name
WikiPathways:WP4686 Leucine, isoleucine and valine metabolism
PathBank:SMP0120838 Glycogenosis, Type VI. Hers Disease
PathBank:SMP0120617 Glycogenosis, Type III. Cori Disease, Debrancher Glycogenosis
PathBank:SMP0120621 Sucrase-Isomaltase Deficiency
PathBank:SMP0000552 Glycogen Synthetase Deficiency
PathBank:SMP0000556 Mucopolysaccharidosis VII. Sly Syndrome
PathBank:SMP0063673 Starch and Sucrose Metabolism
PathBank:SMP0120837 Glycogenosis, Type IV. Amylopectinosis, Anderson Disease
PathBank:SMP0120616 Glycogen Synthetase Deficiency
PathBank:SMP0120620 Mucopolysaccharidosis VII. Sly Syndrome
PathBank:SMP0000555 Glycogenosis, Type VI. Hers Disease
PathBank:SMP0087345 Starch and Sucrose Metabolism
PathBank:SMP0120836 Glycogenosis, Type III. Cori Disease, Debrancher Glycogenosis
PathBank:SMP0120840 Sucrase-Isomaltase Deficiency
PathBank:SMP0120618 Glycogenosis, Type IV. Amylopectinosis, Anderson Disease
PathBank:SMP0000553 Glycogenosis, Type III. Cori Disease, Debrancher Glycogenosis
PathBank:SMP0000557 Sucrase-Isomaltase Deficiency
PathBank:SMP0087252 Starch and Sucrose Metabolism
PathBank:SMP0120835 Glycogen Synthetase Deficiency
PathBank:SMP0120839 Mucopolysaccharidosis VII. Sly Syndrome
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