KEGG   Homo sapiens (human): 1629Help
Entry
1629              CDS       T01001                                 

Gene name
DBT, BCATE2, BCKAD-E2, BCKADE2, BCOADC-E2, E2, E2B
Definition
(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
hsa01100  Metabolic pathways
Module
hsa_M00036  Leucine degradation, leucine => acetoacetate + acetyl-CoA
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)
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)
BRITE hierarchy
SSDB OrthologParalogGFIT
Motif
Pfam: 2-oxoacid_dh Biotin_lipoyl E3_binding HlyD_D23 Toprim_Crpt MtrB
Motif
Other DBs
NCBI-GeneID: 1629
NCBI-ProteinID: NP_001909
OMIM: 248610
HGNC: 2698
Ensembl: ENSG00000137992
Vega: OTTHUMG00000010921
Pharos: P11182(Tbio)
UniProt: P11182
Structure
PDB: 

Position
1p21.2
AA seq 482 aa AA seqDB search
MAAVRMLRTWSRNAGKLICVRYFQTCGNVHVLKPNYVCFFGYPSFKYSHPHHFLKTTAAL
RGQVVQFKLSDIGEGIREVTVKEWYVKEGDTVSQFDSICEVQSDKASVTITSRYDGVIKK
LYYNLDDIAYVGKPLVDIETEALKDSEEDVVETPAVSHDEHTHQEIKGRKTLATPAVRRL
AMENNIKLSEVVGSGKDGRILKEDILNYLEKQTGAILPPSPKVEIMPPPPKPKDMTVPIL
VSKPPVFTGKDKTEPIKGFQKAMVKTMSAALKIPHFGYCDEIDLTELVKLREELKPIAFA
RGIKLSFMPFFLKAASLGLLQFPILNASVDENCQNITYKASHNIGIAMDTEQGLIVPNVK
NVQICSIFDIATELNRLQKLGSVGQLSTTDLTGGTFTLSNIGSIGGTFAKPVIMPPEVAI
GALGSIKAIPRFNQKGEVYKAQIMNVSWSADHRVIDGATMSRFSNLWKSYLENPAFMLLD
LK
NT seq 1449 nt NT seq  +upstreamnt  +downstreamnt
atggctgcagtccgtatgctgagaacctggagcaggaatgcggggaagctgatttgtgtt
cgctattttcaaacatgtggtaatgttcatgttttgaagccaaattatgtgtgtttcttt
ggttatccttcattcaagtatagtcatccacatcacttcctgaaaacaactgctgctctc
cgtggacaggttgttcagttcaagctctcagacattggagaagggattagagaagtaact
gttaaagaatggtatgtaaaagaaggagatacagtgtctcagtttgatagcatctgtgaa
gttcaaagtgataaagcttctgttaccatcactagtcgttatgatggagtcattaaaaaa
ctctattataatctagacgatattgcctatgtggggaagccattagtagacatagaaacg
gaagctttaaaagattcagaagaagatgttgttgaaactcctgcagtgtctcatgatgaa
catacacaccaagagataaagggccgaaaaacactggcaactcctgcagttcgccgtctg
gcaatggaaaacaatattaagctgagtgaagttgttggctcaggaaaagatggcagaata
cttaaagaagatatcctcaactatttggaaaagcagacaggagctatattgcctccttca
cccaaagttgaaattatgccacctccaccaaagccaaaagacatgactgttcctatacta
gtatcaaaacctccggtattcacaggcaaagacaaaacagaacccataaaaggctttcaa
aaagcaatggtcaagactatgtctgcagccctgaagatacctcattttggttattgtgat
gagattgaccttactgaactggttaagctccgagaagaattaaaacccattgcatttgct
cgtggaattaaactctcctttatgcctttcttcttaaaggctgcttccttgggattacta
cagtttcctatccttaacgcttctgtggatgaaaactgccagaatataacatataaggct
tctcataacattgggatagcaatggatactgagcagggtttgattgtccctaatgtgaaa
aatgttcagatctgctctatatttgacatcgccactgaactgaaccgcctccagaaattg
ggctctgtgggtcagctcagcaccactgatcttacaggaggaacatttactctttccaac
attggatcaattggtggtacctttgccaaaccagtgataatgccacctgaagtagccatt
ggggcccttggatcaattaaggccattccccgatttaaccagaaaggagaagtatataag
gcacagataatgaatgtgagctggtcagctgatcacagagttattgatggtgctacaatg
tcacgcttctccaatttgtggaaatcctatttagaaaacccagcttttatgctactagat
ctgaaatga

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