KEGG   Homo sapiens (human): 1629
Entry
1629              CDS       T01001                                 
Symbol
DBT, BCATE2, BCKAD-E2, BCKADE2, BCKDH-E2, BCOADC-E2, E2, E2B
Name
(RefSeq) dihydrolipoamide branched chain transacylase E2
  KO
K09699  2-oxoisovalerate dehydrogenase E2 component (dihydrolipoyl transacylase) [EC:2.3.1.168]
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
    1629 (DBT)
  09105 Amino acid metabolism
   00280 Valine, leucine and isoleucine degradation
    1629 (DBT)
  09108 Metabolism of cofactors and vitamins
   00785 Lipoic acid metabolism
    1629 (DBT)
Enzymes [BR:hsa01000]
 2. Transferases
  2.3  Acyltransferases
   2.3.1  Transferring groups other than aminoacyl groups
    2.3.1.168  dihydrolipoyllysine-residue (2-methylpropanoyl)transferase
     1629 (DBT)
SSDB
Motif
Pfam: 2-oxoacid_dh Biotin_lipoyl E3_binding HlyD_D23
Other DBs
NCBI-GeneID: 1629
NCBI-ProteinID: NP_001909
OMIM: 248610
HGNC: 2698
Ensembl: ENSG00000137992
UniProt: P11182
Structure
LinkDB
Position
1:complement(100186919..100249834)
AA seq 482 aa
MAAVRMLRTWSRNAGKLICVRYFQTCGNVHVLKPNYVCFFGYPSFKYSHPHHFLKTTAAL
RGQVVQFKLSDIGEGIREVTVKEWYVKEGDTVSQFDSICEVQSDKASVTITSRYDGVIKK
LYYNLDDIAYVGKPLVDIETEALKDSEEDVVETPAVSHDEHTHQEIKGRKTLATPAVRRL
AMENNIKLSEVVGSGKDGRILKEDILNYLEKQTGAILPPSPKVEIMPPPPKPKDMTVPIL
VSKPPVFTGKDKTEPIKGFQKAMVKTMSAALKIPHFGYCDEIDLTELVKLREELKPIAFA
RGIKLSFMPFFLKAASLGLLQFPILNASVDENCQNITYKASHNIGIAMDTEQGLIVPNVK
NVQICSIFDIATELNRLQKLGSVSQLSTTDLTGGTFTLSNIGSIGGTFAKPVIMPPEVAI
GALGSIKAIPRFNQKGEVYKAQIMNVSWSADHRVIDGATMSRFSNLWKSYLENPAFMLLD
LK
NT seq 1449 nt   +upstreamnt  +downstreamnt
atggctgcagtccgtatgctgagaacctggagcaggaatgcggggaagctgatttgtgtt
cgctattttcaaacatgtggtaatgttcatgttttgaagccaaattatgtgtgtttcttt
ggttatccttcattcaagtatagtcatccacatcacttcctgaaaacaactgctgctctc
cgtggacaggttgttcagttcaagctctcagacattggagaagggattagagaagtaact
gttaaagaatggtatgtaaaagaaggagatacagtgtctcagtttgatagcatctgtgaa
gttcaaagtgataaagcttctgttaccatcactagtcgttatgatggagtcattaaaaaa
ctctattataatctagacgatattgcctatgtggggaagccattagtagacatagaaacg
gaagctttaaaagattcagaagaagatgttgttgaaactcctgcagtgtctcatgatgaa
catacacaccaagagataaagggccgaaaaacactggcaactcctgcagttcgccgtctg
gcaatggaaaacaatattaagctgagtgaagttgttggctcaggaaaagatggcagaata
cttaaagaagatatcctcaactatttggaaaagcagacaggagctatattgcctccttca
cccaaagttgaaattatgccacctccaccaaagccaaaagacatgactgttcctatacta
gtatcaaaacctccggtattcacaggcaaagacaaaacagaacccataaaaggctttcaa
aaagcaatggtcaagactatgtctgcagccctgaagatacctcattttggttattgtgat
gagattgaccttactgaactggttaagctccgagaagaattaaaacccattgcatttgct
cgtggaattaaactctcctttatgcctttcttcttaaaggctgcttccttgggattacta
cagtttcctatccttaacgcttctgtggatgaaaactgccagaatataacatataaggct
tctcataacattgggatagcaatggatactgagcagggtttgattgtccctaatgtgaaa
aatgttcagatctgctctatatttgacatcgccactgaactgaaccgcctccagaaattg
ggctctgtgagtcagctcagcaccactgatcttacaggaggaacatttactctttccaac
attggatcaattggtggtacctttgccaaaccagtgataatgccacctgaagtagccatt
ggggcccttggatcaattaaggccattccccgatttaaccagaaaggagaagtatataag
gcacagataatgaatgtgagctggtcagctgatcacagagttattgatggtgctacaatg
tcacgcttctccaatttgtggaaatcctatttagaaaacccagcttttatgctactagat
ctgaaatga

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