1DTW

HUMAN BRANCHED-CHAIN ALPHA-KETO ACID DEHYDROGENASE

1DTW の概要

• エントリーDOI: 10.2210/pdb1dtw/pdb
• 分子名称: BRANCHED-CHAIN ALPHA-KETO ACID DEHYDROGENASE ALPHA SUBUNIT, BRANCHED-CHAIN ALPHA-KETO ACID DEHYDROGENASE BETA SUBUNIT, MAGNESIUM ION, ... (6 entities in total)
• 機能のキーワード: thdp-binding fold, branched-chain alpha-keto acid dehydrogenase, oxidoreductase
• 由来する生物種: Homo sapiens (human); 詳細
• 細胞内の位置: Mitochondrion matrix: P12694 P21953
• タンパク質・核酸の鎖数: 2
• 化学式量合計: 84001.18

構造登録者

AEvarsson, A.,Chuang, J.L.,Wynn, R.M.,Turley, S.,Chuang, D.T.,Hol, W.G.J. (登録日: 2000-01-13, 公開日: 2000-03-27, 最終更新日: 2024-02-07)

主引用文献

AEvarsson, A.,Chuang, J.L.,Wynn, R.M.,Turley, S.,Chuang, D.T.,Hol, W.G.
Crystal structure of human branched-chain alpha-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease.
Structure Fold.Des., 8:277-291, 2000

PubMed Abstract: Mutations in components of the extraordinarily large alpha-ketoacid dehydrogenase multienzyme complexes can lead to serious and often fatal disorders in humans, including maple syrup urine disease (MSUD). In order to obtain insight into the effect of mutations observed in MSUD patients, we determined the crystal structure of branched-chain alpha-ketoacid dehydrogenase (E1), the 170 kDa alpha(2)beta(2) heterotetrameric E1b component of the branched-chain alpha-ketoacid dehydrogenase multienzyme complex.

PubMed: 10745006
DOI: 10.1016/S0969-2126(00)00105-2

実験手法

X-RAY DIFFRACTION (2.7 Å)