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KMVA [mitochondrial matrix]
Stable Identifier
R-ALL-113576
Type
Chemical Compound [SimpleEntity]
Compartment
mitochondrial matrix
Synonyms
alpha-keto-beta-methylvalerate, 3-methyl-2-oxovalerate, 3-methyl-2-oxopentanoate
Locations in the PathwayBrowser
for Species:
Homo sapiens
Bos taurus
Caenorhabditis elegans
Canis familiaris
Danio rerio
Dictyostelium discoideum
Drosophila melanogaster
Gallus gallus
Mus musculus
Plasmodium falciparum
Rattus norvegicus
Saccharomyces cerevisiae
Schizosaccharomyces pombe
Sus scrofa
Xenopus tropicalis
Expand all
Metabolism (Bos taurus)
Metabolism of amino acids and derivatives (Bos taurus)
Branched-chain amino acid catabolism (Bos taurus)
BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV (Bos taurus)
BCKDHA:BCKDHB tetramer decarboxylates KIC, KMVA, KIV (Bos taurus)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate <=> leu, ile, or val + alpha-ketoglutarate [BCAT2] (Bos taurus)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
leu, ile, or val + alpha-ketoglutarate <=> a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate [BCAT2] (Bos taurus)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
Metabolism (Caenorhabditis elegans)
Metabolism of amino acids and derivatives (Caenorhabditis elegans)
Branched-chain amino acid catabolism (Caenorhabditis elegans)
BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV (Caenorhabditis elegans)
BCKDHA:BCKDHB tetramer decarboxylates KIC, KMVA, KIV (Caenorhabditis elegans)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate <=> leu, ile, or val + alpha-ketoglutarate [BCAT2] (Caenorhabditis elegans)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
leu, ile, or val + alpha-ketoglutarate <=> a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate [BCAT2] (Caenorhabditis elegans)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
Metabolism (Canis familiaris)
Metabolism of amino acids and derivatives (Canis familiaris)
Branched-chain amino acid catabolism (Canis familiaris)
BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV (Canis familiaris)
BCKDHA:BCKDHB tetramer decarboxylates KIC, KMVA, KIV (Canis familiaris)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate <=> leu, ile, or val + alpha-ketoglutarate [BCAT2] (Canis familiaris)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
leu, ile, or val + alpha-ketoglutarate <=> a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate [BCAT2] (Canis familiaris)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
Metabolism (Danio rerio)
Metabolism of amino acids and derivatives (Danio rerio)
Branched-chain amino acid catabolism (Danio rerio)
BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV (Danio rerio)
BCKDHA:BCKDHB tetramer decarboxylates KIC, KMVA, KIV (Danio rerio)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate <=> leu, ile, or val + alpha-ketoglutarate [BCAT2] (Danio rerio)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
leu, ile, or val + alpha-ketoglutarate <=> a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate [BCAT2] (Danio rerio)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
Metabolism (Dictyostelium discoideum)
Metabolism of amino acids and derivatives (Dictyostelium discoideum)
Branched-chain amino acid catabolism (Dictyostelium discoideum)
BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV (Dictyostelium discoideum)
BCKDHA:BCKDHB tetramer decarboxylates KIC, KMVA, KIV (Dictyostelium discoideum)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate <=> leu, ile, or val + alpha-ketoglutarate [BCAT2] (Dictyostelium discoideum)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
leu, ile, or val + alpha-ketoglutarate <=> a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate [BCAT2] (Dictyostelium discoideum)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
Metabolism (Drosophila melanogaster)
Metabolism of amino acids and derivatives (Drosophila melanogaster)
Branched-chain amino acid catabolism (Drosophila melanogaster)
BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV (Drosophila melanogaster)
BCKDHA:BCKDHB tetramer decarboxylates KIC, KMVA, KIV (Drosophila melanogaster)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate <=> leu, ile, or val + alpha-ketoglutarate [BCAT2] (Drosophila melanogaster)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
leu, ile, or val + alpha-ketoglutarate <=> a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate [BCAT2] (Drosophila melanogaster)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
Metabolism (Gallus gallus)
Metabolism of amino acids and derivatives (Gallus gallus)
Branched-chain amino acid catabolism (Gallus gallus)
BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV (Gallus gallus)
BCKDHA:BCKDHB tetramer decarboxylates KIC, KMVA, KIV (Gallus gallus)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
Disease (Homo sapiens)
Diseases of metabolism (Homo sapiens)
Diseases of branched-chain amino acid catabolism (Homo sapiens)
Maple Syrup Urine Disease (Homo sapiens)
Loss-of-function mutations in BCKDHA or BCKDHB cause MSUD (Homo sapiens)
BCKDHA or BCKDHB loss-of-function mutants don't synthesize BCAA-CoA (Homo sapiens)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
Metabolism (Homo sapiens)
Metabolism of amino acids and derivatives (Homo sapiens)
Branched-chain amino acid catabolism (Homo sapiens)
BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV (Homo sapiens)
BCKDHA:BCKDHB tetramer decarboxylates KIC, KMVA, KIV (Homo sapiens)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate <=> leu, ile, or val + alpha-ketoglutarate [BCAT2] (Homo sapiens)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
leu, ile, or val + alpha-ketoglutarate <=> a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate [BCAT2] (Homo sapiens)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
Metabolism (Mus musculus)
Metabolism of amino acids and derivatives (Mus musculus)
Branched-chain amino acid catabolism (Mus musculus)
BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV (Mus musculus)
BCKDHA:BCKDHB tetramer decarboxylates KIC, KMVA, KIV (Mus musculus)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate <=> leu, ile, or val + alpha-ketoglutarate [BCAT2] (Mus musculus)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
leu, ile, or val + alpha-ketoglutarate <=> a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate [BCAT2] (Mus musculus)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
Metabolism (Plasmodium falciparum)
Metabolism of amino acids and derivatives (Plasmodium falciparum)
Branched-chain amino acid catabolism (Plasmodium falciparum)
BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV (Plasmodium falciparum)
BCKDHA:BCKDHB tetramer decarboxylates KIC, KMVA, KIV (Plasmodium falciparum)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
Metabolism (Rattus norvegicus)
Metabolism of amino acids and derivatives (Rattus norvegicus)
Branched-chain amino acid catabolism (Rattus norvegicus)
BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV (Rattus norvegicus)
BCKDHA:BCKDHB tetramer decarboxylates KIC, KMVA, KIV (Rattus norvegicus)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate <=> leu, ile, or val + alpha-ketoglutarate [BCAT2] (Rattus norvegicus)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
leu, ile, or val + alpha-ketoglutarate <=> a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate [BCAT2] (Rattus norvegicus)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
Metabolism (Saccharomyces cerevisiae)
Metabolism of amino acids and derivatives (Saccharomyces cerevisiae)
Branched-chain amino acid catabolism (Saccharomyces cerevisiae)
a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate <=> leu, ile, or val + alpha-ketoglutarate [BCAT2] (Saccharomyces cerevisiae)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
leu, ile, or val + alpha-ketoglutarate <=> a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate [BCAT2] (Saccharomyces cerevisiae)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
Metabolism (Schizosaccharomyces pombe)
Metabolism of amino acids and derivatives (Schizosaccharomyces pombe)
Branched-chain amino acid catabolism (Schizosaccharomyces pombe)
a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate <=> leu, ile, or val + alpha-ketoglutarate [BCAT2] (Schizosaccharomyces pombe)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
leu, ile, or val + alpha-ketoglutarate <=> a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate [BCAT2] (Schizosaccharomyces pombe)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
Metabolism (Sus scrofa)
Metabolism of amino acids and derivatives (Sus scrofa)
Branched-chain amino acid catabolism (Sus scrofa)
BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV (Sus scrofa)
BCKDHA:BCKDHB tetramer decarboxylates KIC, KMVA, KIV (Sus scrofa)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate <=> leu, ile, or val + alpha-ketoglutarate [BCAT2] (Sus scrofa)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
leu, ile, or val + alpha-ketoglutarate <=> a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate [BCAT2] (Sus scrofa)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
Metabolism (Xenopus tropicalis)
Metabolism of amino acids and derivatives (Xenopus tropicalis)
Branched-chain amino acid catabolism (Xenopus tropicalis)
BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV (Xenopus tropicalis)
BCKDHA:BCKDHB tetramer decarboxylates KIC, KMVA, KIV (Xenopus tropicalis)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate <=> leu, ile, or val + alpha-ketoglutarate [BCAT2] (Xenopus tropicalis)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
leu, ile, or val + alpha-ketoglutarate <=> a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate [BCAT2] (Xenopus tropicalis)
KIC, KMVA, KIV [mitochondrial matrix]
KMVA [mitochondrial matrix]
External Reference Information
External Reference
(S)-3-methyl-2-oxovalerate [ChEBI:35146]
Participates
as a member of
KIC, KMVA, KIV [mitochondrial matrix]
Other forms of this molecule
KMVA [cytosol]
Cross References
AraCyc
2-KETO-3-METHYL-VALERATE
CAS
1460-34-0
COMPOUND
C03465
PubChem Compound
6857401
ZINC
ZINC000001532715
KEGG Gene
C00671
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