PDB entry 1x7z

View 1x7z on RCSB PDB site
Description: Crystal structure of the human mitochondrial branched-chain alpha-ketoacid dehydrogenase
Class: oxidoreductase
Keywords: oxidoreductase,ketoacid dehydrogenase, branched-chain, multi-enzyme complex, acylation, oxidative decarboxylation maple syrup urine disease, thiamin diphosphate, phosphorylation, flavoprotein, oxidoreductase
Deposited on 2004-08-16, released 2004-11-23
The last revision prior to the SCOPe 2.08 freeze date was dated 2011-07-13, with a file datestamp of 2011-05-08.
Experiment type: XRAY
Resolution: 1.72 Å
R-factor: 0.154
AEROSPACI score: 0.57 (click here for full SPACI score report)

Chains and heterogens:

  • Chain 'A':
    Compound: 2-oxoisovalerate dehydrogenase alpha subunit
    Species: Homo sapiens [TaxId:9606]
    Gene: BCKDHA
    Database cross-references and differences (RAF-indexed):
    • Uniprot P12694 (Start-399)
      • engineered (291)
    Domains in SCOPe 2.08: d1x7za_
  • Chain 'B':
    Compound: 2-oxoisovalerate dehydrogenase beta subunit
    Species: Homo sapiens [TaxId:9606]
    Gene: BCKDHB
    Database cross-references and differences (RAF-indexed): Domains in SCOPe 2.08: d1x7zb1, d1x7zb2
  • Heterogens: K, MN, CL, TDP, GOL, HOH

PDB Chain Sequences:

  • Chain 'A':
    Sequence, based on SEQRES records: (download) 
    >1x7zA (A:)
sslddkpqfpgasaefidklefiqpnvisgipiyrvmdrqgqiinpsedphlpkekvlkl
yksmtllntmdrilyesqrqgrisfymtnygeegthvgsaaaldntdlvfgqyreagvlm
yrdyplelfmaqcygnisdlgkgrqmpvhygckerhfvtissplatqipqavgaayaakr
ananrvvicyfgegaasegdahagfnfaatlecpiiffcrnngyaistptseqyrgdgia
argpgygimsirvdgndvfavynatkearrravaenqpflieamtyrighhdtsddssay
rsvdevnywdkqdhpisrlrhyllsqgwwdeeqekawrkqsrrkvmeafeqaerkpkpnp
nllfsdvyqempaqlrkqqeslarhlqtygehypldhfdk

    Sequence, based on observed residues (ATOM records): (download) 
    >1x7zA (A:)
kpqfpgasaefidklefiqpnvisgipiyrvmdrqgqiinpsedphlpkekvlklyksmt
llntmdrilyesqrqgrisfymtnygeegthvgsaaaldntdlvfgqyreagvlmyrdyp
lelfmaqcygnisdlgkgrqmpvhygckerhfvtissplatqipqavgaayaakrananr
vvicyfgegaasegdahagfnfaatlecpiiffcrnngyaistptseqyrgdgiaargpg
ygimsirvdgndvfavynatkearrravaenqpflieamtyrighhdtsddssayrvnyw
dkqdhpisrlrhyllsqgwwdeeqekawrkqsrrkvmeafeqaerkpkpnpnllfsdvyq
empaqlrkqqeslarhlqtygehypldhfdk

  • Chain 'B':
    Sequence, based on SEQRES records: (download) 
    >1x7zB (B:)
vahftfqpdpepreygqtqkmnlfqsvtsaldnslakdptavifgedvafggvfrctvgl
rdkygkdrvfntplceqgivgfgigiavtgataiaeiqfadyifpafdqivneaakyryr
sgdlfncgsltirspwgcvghgalyhsqspeaffahcpgikvviprspfqakglllscie
dknpciffepkilyraaaeevpiepyniplsqaeviqegsdvtlvawgtqvhvirevasm
akeklgvscevidlrtiipwdvdticksviktgrllisheapltggfaseisstvqeecf
lnleapisrvcgydtpfphifepfyipdkwkcydalrkminy

    Sequence, based on observed residues (ATOM records): (download) 
    >1x7zB (B:)
eygqtqkmnlfqsvtsaldnslakdptavifgedvafggvfrctvglrdkygkdrvfntp
lceqgivgfgigiavtgataiaeiqfadyifpafdqivneaakyryrsgdlfncgsltir
spwgcvghgalyhsqspeaffahcpgikvviprspfqakglllsciedknpciffepkil
yraaaeevpiepyniplsqaeviqegsdvtlvawgtqvhvirevasmakeklgvscevid
lrtiipwdvdticksviktgrllisheapltggfaseisstvqeecflnleapisrvcgy
dtpfphifepfyipdkwkcydalrkminy