| Entry |
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| Symbol |
BCKDHA, BCKDE1A, MSU, MSUD1, MSUD1A, OVD1A
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| Name |
(RefSeq) 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform 1 precursor
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| KO |
| K00166 | 2-oxoisovalerate dehydrogenase E1 component subunit alpha [EC:1.2.4.4] |
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| Organism |
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| Pathway |
| hsa00280 | Valine, leucine and isoleucine degradation |
| hsa01210 | 2-Oxocarboxylic acid metabolism |
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| Module |
| hsa_M00036 | Leucine degradation, leucine => acetoacetate + acetyl-CoA |
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| Network |
nt06024 Valine, leucine and isoleucine degradation nt06032 Lipoic acid metabolism |
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| Element |
| N01619 | Branched-chain amino acids degradation 2 |
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| Disease |
| H00172 | Maple syrup urine disease |
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| Brite |
KEGG Orthology (KO) [BR:hsa00001]
09100 Metabolism
09101 Carbohydrate metabolism
00640 Propanoate metabolism
593 (BCKDHA)
09105 Amino acid metabolism
00280 Valine, leucine and isoleucine degradation
593 (BCKDHA)
09108 Metabolism of cofactors and vitamins
00785 Lipoic acid metabolism
593 (BCKDHA)
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)
593 (BCKDHA)
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| SSDB |
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| Motif |
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| Other DBs |
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| Structure |
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| LinkDB |
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| Position |
19:41397818..41425002
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| AA seq |
445 aa
MAVAIAAARVWRLNRGLSQAALLLLRQPGARGLARSHPPRQQQQFSSLDDKPQFPGASAE
FIDKLEFIQPNVISGIPIYRVMDRQGQIINPSEDPHLPKEKVLKLYKSMTLLNTMDRILY
ESQRQGRISFYMTNYGEEGTHVGSAAALDNTDLVFGQYREAGVLMYRDYPLELFMAQCYG
NISDLGKGRQMPVHYGCKERHFVTISSPLATQIPQAVGAAYAAKRANANRVVICYFGEGA
ASEGDAHAGFNFAATLECPIIFFCRNNGYAISTPTSEQYRGDGIAARGPGYGIMSIRVDG
NDVFAVYNATKEARRRAVAENQPFLIEAMTYRIGHHSTSDDSSAYRSVDEVNYWDKQDHP
ISRLRHYLLSQGWWDEEQEKAWRKQSRRKVMEAFEQAERKPKPNPNLLFSDVYQEMPAQL
RKQQESLARHLQTYGEHYPLDHFDK |
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| NT seq |
1338 nt +upstreamnt +downstreamnt
atggcggtagcgatcgctgcagcgagggtctggcggctaaaccgtggtttgagccaggct
gccctcctgctgctgcggcagcctggggctcggggactggctagatctcacccccccagg
cagcagcagcagttttcatctctggatgacaagccccagttcccaggggcctcggcggag
tttatagataagttggaattcatccagcccaacgtcatctctggaatccccatctaccgc
gtcatggaccggcaaggccagatcatcaaccccagcgaggacccccacctgccgaaggag
aaggtgctgaagctctacaagagcatgacactgcttaacaccatggaccgcatcctctat
gagtctcagcggcagggccggatctccttctacatgaccaactatggtgaggagggcacg
cacgtggggagtgccgccgccctggacaacacggacctggtgtttggccagtaccgggag
gcaggtgtgctgatgtatcgggactaccccctggaactattcatggcccagtgctatggc
aacatcagtgacttgggcaaggggcgccagatgcctgtccactacggctgcaaggaacgc
cacttcgtcactatctcctctccactggccacgcagatccctcaggcggtgggggcggcg
tacgcagccaagcgggccaatgccaacagggtcgtcatctgttacttcggcgagggggca
gccagtgagggggacgcccatgccggcttcaacttcgctgccacacttgagtgccccatc
atcttcttctgccggaacaatggctacgccatctccacgcccacctctgagcagtatcgc
ggcgatggcattgcagcacgaggccccgggtatggcatcatgtcaatccgcgtggatggt
aatgatgtgtttgccgtatacaacgccacaaaggaggcccgacggcgggctgtggcagag
aaccagcccttcctcatcgaggccatgacctacaggatcgggcaccacagcaccagtgac
gacagttcagcgtaccgctcggtggatgaggtcaattactgggataaacaggaccacccc
atctcccggctgcggcactatctgctgagccaaggctggtgggatgaggagcaggagaag
gcctggaggaagcagtcccgcaggaaggtgatggaggcctttgagcaggccgagcggaag
cccaaacccaaccccaacctactcttctcagacgtgtatcaggagatgcccgcccagctc
cgcaagcagcaggagtctctggcccgccacctgcagacctacggggagcactacccactg
gatcacttcgataagtga |
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