Crystal structure of human branched-chain α-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease

Structure

Volumn 8, Issue 3, 2000, Pages 277-291

  Ævarsson, Arnthor ^a   Chuang, Jacinta L ^b   Max Wynn, R ^b   Turley, Stewart ^c   Chuang, David T ^b   Hol, Wim G J ^a,b  

^a UNIVERSITY OF WASHINGTON SCHOOL OF MEDICINE   (United States)

^b UNIVERSITY OF TEXAS AT DALLAS   (United States)

^c UNIVERSITY OF WASHINGTON   (United States)

Author keywords

Maple syrup Urine disease; Mennonite; Thiamin diphosphate; ketoacid dehydrogenase; ketoacid dehydrogenase multienzyme complex

Indexed keywords

2 OXOACID; MULTIENZYME COMPLEX; OXIDOREDUCTASE; POTASSIUM ION; PROTEIN SUBUNIT;

AMINO ACID SEQUENCE; ARTICLE; BINDING SITE; CRYSTAL STRUCTURE; ENZYME DEFICIENCY; HUMAN; MAPLE SYRUP URINE DISEASE; MISSENSE MUTATION; PRIORITY JOURNAL; PROTEIN BINDING; PROTEIN CONFORMATION; PROTEIN PROTEIN INTERACTION; PROTEIN STABILITY; PROTEIN STRUCTURE; SEQUENCE ANALYSIS;

ACER;

EID: 0034653468     PISSN: 09692126     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0969-2126(00)00105-2     Document Type: Article

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