Pathogenic mutations differentially affect the catalytic activities of the human B12-processing chaperone CblC and increase futile redox cycling

Journal of Biological Chemistry

Volumn 290, Issue 18, 2015, Pages 11393-11402

  Gherasim, Carmen ^a   Ruetz, Markus ^a   Li, Zhu ^a   Hudolin, Stephanie ^a   Banerjee, Ruma ^a  

^a UNIVERSITY OF MICHIGAN HEALTH SYSTEM   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

BIOCHEMISTRY; BIOLOGY;

DEALKYLATION; GLUTATHIONES; PATHOGENIC MUTATION; REDOX CYCLING;

CATALYST ACTIVITY;

ARGININE; CHAPERONE; COBALAMIN; GLUTATHIONE; GLUTATHIONE DISULFIDE; PROTEIN CBLC; REACTIVE OXYGEN METABOLITE; UNCLASSIFIED DRUG; CBL PROTEIN; CBLC PROTEIN, HUMAN; CYANOCOBALAMIN; HYDROXOCOBALAMIN; NITRILE;

ARTICLE; BINDING AFFINITY; CATALYSIS; CBLC GENE; COBALAMIN C DISEASE; CONCENTRATION (PARAMETERS); CONTROLLED STUDY; CYANATION; DEALKYLATION; DISEASE COURSE; GENE; HUMAN; HUMAN CELL; MISSENSE MUTATION; MOLECULAR PATHOLOGY; MUTANT; OXIDATION REDUCTION REACTION; OXIDATIVE STRESS; PRIORITY JOURNAL; PROTEIN BINDING; PROTEIN DEFECT; PROTEIN STABILITY; PROTEIN STRUCTURE; ALKYLATION; ANALOGS AND DERIVATIVES; BIOCATALYSIS; CHEMICAL STRUCTURE; CHEMISTRY; DRUG EFFECTS; GENETICS; INBORN ERROR OF METABOLISM; METABOLISM; PROTEIN TERTIARY STRUCTURE;

ALKYLATION; ARGININE; BIOCATALYSIS; GLUTATHIONE; HUMANS; METABOLISM, INBORN ERRORS; MODELS, MOLECULAR; MOLECULAR CHAPERONES; MUTATION, MISSENSE; NITRILES; OXIDATION-REDUCTION; PROTEIN STABILITY; PROTEIN STRUCTURE, TERTIARY; PROTO-ONCOGENE PROTEINS C-CBL; REACTIVE OXYGEN SPECIES; VITAMIN B 12;

EID: 84929483900     PISSN: 00219258     EISSN: 1083351X     Source Type: Journal    
DOI: 10.1074/jbc.M115.637132     Document Type: Article

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