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Record Information
Version5.0
StatusExpected but not Quantified
Creation Date2006-05-22 14:17:36 UTC
Update Date2021-09-14 15:47:57 UTC
HMDB IDHMDB0002091
Secondary Accession Numbers
  • HMDB02091
Metabolite Identification
Common NameIsovalerylglucuronide
DescriptionIsovaleryl 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 ).
Structure
Data?1582752228
Synonyms
ValueSource
Isovaleryl-beta-D-glucuronideHMDB
Isovaleryl-beta-delta-glucuronideHMDB
Chemical FormulaC11H18O8
Average Molecular Weight278.2558
Monoisotopic Molecular Weight278.100167552
IUPAC Name(2S,3S,4S,5R,6S)-3,4,5-trihydroxy-6-[(3-methylbutanoyl)oxy]oxane-2-carboxylic acid
Traditional Nameisovalerylglucuronide
CAS Registry Number88070-93-3
SMILES
CC(C)CC(=O)O[C@@H]1O[C@@H]([C@@H](O)[C@H](O)[C@H]1O)C(O)=O
InChI Identifier
InChI=1S/C11H18O8/c1-4(2)3-5(12)18-11-8(15)6(13)7(14)9(19-11)10(16)17/h4,6-9,11,13-15H,3H2,1-2H3,(H,16,17)/t6-,7-,8+,9-,11+/m0/s1
InChI KeyVOJAALAAOYUSCT-ZCLKDUABSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as o-glucuronides. These are glucuronides in which the aglycone is linked to the carbohydrate unit through an O-glycosidic bond.
KingdomOrganic compounds
Super ClassOrganic oxygen compounds
ClassOrganooxygen compounds
Sub ClassCarbohydrates and carbohydrate conjugates
Direct ParentO-glucuronides
Alternative Parents
Substituents
  • 1-o-glucuronide
  • O-glucuronide
  • Beta-hydroxy acid
  • Fatty acid ester
  • Dicarboxylic acid or derivatives
  • Hydroxy acid
  • Monosaccharide
  • Fatty acyl
  • Oxane
  • Pyran
  • Carboxylic acid ester
  • Secondary alcohol
  • Oxacycle
  • Acetal
  • Carboxylic acid
  • Polyol
  • Carboxylic acid derivative
  • Organoheterocyclic compound
  • Alcohol
  • Hydrocarbon derivative
  • Organic oxide
  • Carbonyl group
  • Aliphatic heteromonocyclic compound
Molecular FrameworkAliphatic heteromonocyclic compounds
External DescriptorsNot Available
Ontology
Physiological effectNot Available
Disposition
Process
Role
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Experimental Chromatographic PropertiesNot Available
Predicted Molecular Properties
Predicted Chromatographic Properties
Spectra
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biospecimen Locations
  • Blood
  • Urine
Tissue Locations
  • Kidney
  • Liver
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodExpected but not QuantifiedNot QuantifiedNot AvailableNot AvailableNormal
      Not Available
details
UrineExpected but not QuantifiedNot QuantifiedNot AvailableNot AvailableNormal
      Not Available
details
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
Phenol Explorer Compound IDNot Available
FooDB IDFDB022841
KNApSAcK IDNot Available
Chemspider ID121058
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
METLIN ID6482
PubChem Compound137383
PDB IDNot Available
ChEBI IDNot Available
Food Biomarker OntologyNot Available
VMH IDNot Available
MarkerDB IDNot Available
Good Scents IDNot Available
References
Synthesis ReferenceNot Available
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Dorland L, Duran M, Wadman SK, Niederwieser A, Bruinvis L, Ketting D: Isovalerylglucuronide, a new urinary metabolite in isovaleric acidemia. Identification problems due to rearrangement reactions. Clin Chim Acta. 1983 Oct 31;134(1-2):77-83. [PubMed:6652913 ]
  2. Hine DG, Tanaka K: The identification and the excretion pattern of isovaleryl glucuronide in the urine of patients with isovaleric acidemia. Pediatr Res. 1984 Jun;18(6):508-12. [PubMed:6547525 ]

Only showing the first 10 proteins. There are 18 proteins in total.

Enzymes

General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
UDPGTs are of major importance in the conjugation and subsequent elimination of potentially toxic xenobiotics and endogenous compounds. This isozyme has glucuronidating capacity with steroid substrates such as 5-beta-androstane 3-alpha,17-beta-diol, estradiol, ADT, eugenol and bile acids. Only isoform 1 seems to be active.
Gene Name:
UGT2B28
Uniprot ID:
Q9BY64
Molecular weight:
38742.9
General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
UDPGTs are of major importance in the conjugation and subsequent elimination of potentially toxic xenobiotics and endogenous compounds. This isozyme is active on polyhydroxylated estrogens (such as estriol, 4-hydroxyestrone and 2-hydroxyestriol) and xenobiotics (such as 4-methylumbelliferone, 1-naphthol, 4-nitrophenol, 2-aminophenol, 4-hydroxybiphenyl and menthol). It is capable of 6 alpha-hydroxyglucuronidation of hyodeoxycholic acid.
Gene Name:
UGT2B4
Uniprot ID:
P06133
Molecular weight:
60512.035
General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
UDPGT is of major importance in the conjugation and subsequent elimination of potentially toxic xenobiotics and endogenous compounds. This isoform glucuronidates bilirubin IX-alpha to form both the IX-alpha-C8 and IX-alpha-C12 monoconjugates and diconjugate.
Gene Name:
UGT1A4
Uniprot ID:
P22310
Molecular weight:
60024.535
General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
UDPGT is of major importance in the conjugation and subsequent elimination of potentially toxic xenobiotics and endogenous compounds. Its unique specificity for 3,4-catechol estrogens and estriol suggests it may play an important role in regulating the level and activity of these potent and active estrogen metabolites. Is also active with androsterone, hyodeoxycholic acid and tetrachlorocatechol (in vitro).
Gene Name:
UGT2B7
Uniprot ID:
P16662
Molecular weight:
60720.15
General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
UDPGTs are of major importance in the conjugation and subsequent elimination of potentially toxic xenobiotics and endogenous compounds. This isozyme displays activity toward several classes of xenobiotic substrates, including simple phenolic compounds, 7-hydroxylated coumarins, flavonoids, anthraquinones, and certain drugs and their hydroxylated metabolites. It also catalyzes the glucuronidation of endogenous estrogens and androgens.
Gene Name:
UGT2B15
Uniprot ID:
P54855
Molecular weight:
61035.815
General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
UDP-glucuronosyltransferases catalyze phase II biotransformation reactions in which lipophilic substrates are conjugated with glucuronic acid to increase water solubility and enhance excretion. They are of major importance in the conjugation and subsequent elimination of potentially toxic xenobiotics and endogenous compounds. Active on odorants and seems to be involved in olfaction; it could help clear lipophilic odorant molecules from the sensory epithelium.
Gene Name:
UGT2A1
Uniprot ID:
Q9Y4X1
Molecular weight:
60771.605
General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
UDPGT is of major importance in the conjugation and subsequent elimination of potentially toxic xenobiotics and endogenous compounds. This isoform glucuronidates bilirubin IX-alpha to form both the IX-alpha-C8 and IX-alpha-C12 monoconjugates and diconjugate. Is also able to catalyze the glucuronidation of 17beta-estradiol, 17alpha-ethinylestradiol, 1-hydroxypyrene, 4-methylumbelliferone, 1-naphthol, paranitrophenol, scopoletin, and umbelliferone.
Gene Name:
UGT1A1
Uniprot ID:
P22309
Molecular weight:
59590.91
General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
UDPGT is of major importance in the conjugation and subsequent elimination of potentially toxic xenobiotics and endogenous compounds. This isoform has specificity for phenols.
Gene Name:
UGT1A9
Uniprot ID:
O60656
Molecular weight:
59940.495
General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
UDPGT is of major importance in the conjugation and subsequent elimination of potentially toxic xenobiotics and endogenous compounds.
Gene Name:
UGT1A3
Uniprot ID:
P35503
Molecular weight:
60337.835
General function:
Involved in transferase activity, transferring hexosyl groups
Specific function:
UDPGT is of major importance in the conjugation and subsequent elimination of potentially toxic xenobiotics and endogenous compounds. The major substrates of this isozyme are eugenol > 4-methylumbelliferone > dihydrotestosterone (DHT) > androstane-3-alpha,17-beta-diol (3-alpha-diol) > testosterone > androsterone (ADT).
Gene Name:
UGT2B17
Uniprot ID:
O75795
Molecular weight:
61094.915

Only showing the first 10 proteins. There are 18 proteins in total.