Homo sapiens (human): 594
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Entry
594 CDS
T01001
Symbol
BCKDHB, BCKDE1B, BCKDH_E1-beta, E1B, MSUD1B, OVD1B
Name
(RefSeq) branched chain keto acid dehydrogenase E1 subunit beta
KO
K00167
2-oxoisovalerate dehydrogenase E1 component subunit beta [EC:
1.2.4.4
]
Organism
hsa
Homo sapiens (human)
Pathway
hsa00280
Valine, leucine and isoleucine degradation
hsa00640
Propanoate metabolism
hsa00785
Lipoic acid metabolism
hsa01100
Metabolic pathways
hsa01210
2-Oxocarboxylic acid metabolism
Module
hsa_M00036
Leucine degradation, leucine => acetoacetate + acetyl-CoA
Network
nt06024
Valine, leucine and isoleucine degradation
nt06032
Lipoic acid metabolism
Element
N01619
Branched-chain amino acids degradation 2
Disease
H00172
Maple syrup urine disease
Brite
KEGG Orthology (KO) [BR:
hsa00001
]
09100 Metabolism
09101 Carbohydrate metabolism
00640 Propanoate metabolism
594 (BCKDHB)
09105 Amino acid metabolism
00280 Valine, leucine and isoleucine degradation
594 (BCKDHB)
09108 Metabolism of cofactors and vitamins
00785 Lipoic acid metabolism
594 (BCKDHB)
Enzymes [BR:
hsa01000
]
1. Oxidoreductases
1.2 Acting on the aldehyde or oxo group of donors
1.2.4 With a disulfide as acceptor
1.2.4.4 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring)
594 (BCKDHB)
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Motif
Pfam:
Transket_pyr
Transketolase_C
Motif
Other DBs
NCBI-GeneID:
594
NCBI-ProteinID:
NP_000047
OMIM:
248611
HGNC:
987
Ensembl:
ENSG00000083123
Pharos:
P21953
(Tbio)
UniProt:
P21953
A0A140VKB3
Structure
PDB
PDBj
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All DBs
Position
6:80106610..80466676
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AA seq
392 aa
AA seq
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MAVVAAAAGWLLRLRAAGAEGHWRRLPGAGLARGFLHPAATVEDAAQRRQVAHFTFQPDP
EPREYGQTQKMNLFQSVTSALDNSLAKDPTAVIFGEDVAFGGVFRCTVGLRDKYGKDRVF
NTPLCEQGIVGFGIGIAVTGATAIAEIQFADYIFPAFDQIVNEAAKYRYRSGDLFNCGSL
TIRSPWGCVGHGALYHSQSPEAFFAHCPGIKVVIPRSPFQAKGLLLSCIEDKNPCIFFEP
KILYRAAAEEVPIEPYNIPLSQAEVIQEGSDVTLVAWGTQVHVIREVASMAKEKLGVSCE
VIDLRTIIPWDVDTICKSVIKTGRLLISHEAPLTGGFASEISSTVQEECFLNLEAPISRV
CGYDTPFPHIFEPFYIPDKWKCYDALRKMINY
NT seq
1179 nt
NT seq
+upstream
nt +downstream
nt
atggcggttgtagcggcggctgccggctggctactcaggctcagggcggcaggggctgag
gggcactggcgtcggcttcctggcgcggggctggcgcggggctttttgcaccccgccgcg
actgtcgaggatgcggcccagaggcggcaggtggctcattttactttccagccagatccg
gagccccgggagtacgggcaaactcagaaaatgaatcttttccagtctgtaacaagtgcc
ttggataactcattggccaaagatcctactgcagtaatatttggtgaagatgttgccttt
ggtggagtctttagatgcactgttggcttgcgagacaaatatggaaaagatagagttttt
aataccccattgtgtgaacaaggaattgttggatttggaatcggaattgcggtcactgga
gctactgccattgcggaaattcagtttgcagattatattttccctgcatttgatcagatt
gttaatgaagctgccaagtatcgctatcgctctggggatctttttaactgtggaagcctc
actatccggtccccttggggctgtgttggtcatggggctctctatcattctcagagtcct
gaagcattttttgcccattgcccaggaatcaaggtggttatacccagaagccctttccag
gccaaaggacttcttttgtcatgcatagaggataaaaatccttgtatattttttgaacct
aaaatactttacagggcagcagcggaagaagtccctatagaaccatacaacatcccactg
tcccaggccgaagtcatacaggaagggagtgatgttactctagttgcctggggcactcag
gttcatgtgatccgagaggtagcttccatggcaaaagaaaagcttggagtgtcttgtgaa
gtcattgatctgaggactataataccttgggatgtggacacaatttgtaagtctgtgatc
aaaacagggcgactgctaatcagtcacgaggctcccttgacaggcggctttgcatcggaa
atcagctctacagttcaggaggaatgtttcttgaacctagaggctcctatatcaagagta
tgtggttatgacacaccatttcctcacatttttgaaccattctacatcccagacaaatgg
aagtgttatgatgcccttcgaaaaatgatcaactattga
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