3-METHYL-2-OXOPENTANOIC ACID, SODIUM SALT

Relevant Data

Food Additives Approved in the United States

Flavouring Substances Approved by European Union:

  • 3-Methyl-2-oxovaleric acid [show]

General Information

Synonyms: 3-METHYL-2-OXOVALERIC ACID, SODIUM SALT, SODIUM 3-METHYL-2-OXOPENTANOATE
Chemical Names: SODIUM 3-METHYL-2-OXOVALERATE
CAS number: 1460-34-0
JECFA number: 632
FEMA number: 3870
Functional Class: Flavouring Agent
FLAVOURING_AGENT

From apps.who.int

Evaluations

Evaluation year: 1999
ADI: No safety concern at current levels of intake when used as a flavouring agent
Report: TRS 896-JECFA 53/67
Tox Monograph: FAS 44-JECFA 53/229
Specification: FAO JECFA Monographs 4/ . R (2007) (SODIUM 3-METHYL-2-OXOPENTANOATE)

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Toxicity Profile

Route of ExposureNone
Mechanism of Toxicity3-Methyl-2-oxovaleric acid is produced from isoleucine by cytosolic branched chain aminotransferase 1, whereupon it is further degraded by branched chain keto acid dehydrogenase E1 to 2-methyl-1-hydroxybutyl-ThPP. It is used as a clinical marker for maple syrup urine disease (MSUD). MSUD is caused by a deficiency of the branched-chain -keto acid dehydrogenase complex resulting in an accumulation of branched-chain amino acids and the corresponding -keto-and -hydroxy acids in blood, urine and cerebrospinal fluid causing neurological damage and mental retardation.
MetabolismNone
Toxicity ValuesNone
Lethal DoseNone
Carcinogenicity (IARC Classification)No indication of carcinogenicity to humans (not listed by IARC).
Minimum Risk LevelNone
Health EffectsChronically high levels of 3-methyl-2-oxovaleric acid are associated with Maple Syrup Urine Disease.
TreatmentNone
Reference
  1. Sreekumar A, Poisson LM, Rajendiran TM, Khan AP, Cao Q, Yu J, Laxman B, Mehra R, Lonigro RJ, Li Y, Nyati MK, Ahsan A, Kalyana-Sundaram S, Han B, Cao X, Byun J, Omenn GS, Ghosh D, Pennathur S, Alexander DC, Berger A, Shuster JR, Wei JT, Varambally S, Beecher C, Chinnaiyan AM: Metabolomic profiles delineate potential role for sarcosine in prostate cancer progression. Nature. 2009 Feb 12;457(7231):910-4. doi: 10.1038/nature07762.[19212411 ]
  2. Wendel U, Even G, Langenbeck U, Schadewaldt P, Hummel W: Determination of (S)- and (R)-2-oxo-3-methylvaleric acid in plasma of patients with maple syrup urine disease. Clin Chim Acta. 1992 Jun 15;208(1-2):85-91.[1638756 ]
  3. Przyrembel H, Bremer HJ, Duran M, Bruinvis L, Ketting D, Wadman SK, Baumgartner R, Irle U, Bachmann C: Propionyl-CoA carboxylase deficiency with overflow of metabolites of isoleucine catabolism at all levels. Eur J Pediatr. 1979 Jan 18;130(1):1-14.[759179 ]

From T3DB