Enzymatic defects underlying hereditary glutamate cysteine ligase deficiency are mitigated by association of the catalytic and regulatory subunits

Biochemistry

Volumn 50, Issue 29, 2011, Pages 6508-6517

  Willis, Melanie Neely ^a   Liu, Yilin ^a   Biterova, Ekaterina I ^a   Simpson, Melanie A ^a   Kim, Heejeong ^a   Lee, Jaekwon ^a   Barycki, Joseph J ^a  

^a UNIVERSITY OF NEBRASKA LINCOLN   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CATALYTIC SUBUNITS; COFACTORS; EMBRYONIC FIBROBLASTS; ENZYMATIC ACTIVITIES; ENZYMATIC FUNCTIONS; GLUTAMATE-CYSTEINE LIGASE; GLUTATHIONES; HETERODIMER FORMATION; HETERODIMERS; IN-VITRO; IN-VIVO; KINETIC CHARACTERIZATION; KINETIC CONSTANT; MODEL SYSTEM; RATE-LIMITING STEPS; RECESSIVE TRAITS; REDOX BUFFER; REGULATORY SUBUNITS; TRANSFECTANTS; WILD TYPES;

CELL CULTURE; ENZYME ACTIVITY; MAMMALS; YEAST;

AMINO ACIDS;

GLUTAMATE CYSTEINE LIGASE; GLUTATHIONE; HETERODIMER;

ANIMAL CELL; ARTICLE; BACTERIAL STRAIN; CONTROLLED STUDY; EMBRYO; ENZYME ACTIVE SITE; ENZYME ACTIVITY; ENZYME DEFICIENCY; FIBROBLAST; IN VITRO STUDY; IN VIVO STUDY; MISSENSE MUTATION; MOUSE; NONHUMAN; PRIORITY JOURNAL; REGULATORY MECHANISM; SACCHAROMYCES CEREVISIAE; TRANSIENT TRANSFECTION; WILD TYPE;

ANIMALS; CATALYTIC DOMAIN; ENZYME STABILITY; GLUTAMATE-CYSTEINE LIGASE; GLUTATHIONE; HOMOZYGOTE; HUMANS; KINETICS; MICE; MODELS, BIOLOGICAL; MUTANT PROTEINS; MUTATION; PROTEIN BINDING; PROTEIN STRUCTURE, SECONDARY; RECOMBINANT PROTEINS; SACCHAROMYCES CEREVISIAE; STRUCTURAL HOMOLOGY, PROTEIN; TEMPERATURE;

MUS; SACCHAROMYCES CEREVISIAE;

EID: 79960499718     PISSN: 00062960     EISSN: 15204995     Source Type: Journal    
DOI: 10.1021/bi200708w     Document Type: Article

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