Disease-proportional proteasomal degradation of missense dystrophins

Proceedings of the National Academy of Sciences of the United States of America

Volumn 112, Issue 40, 2015, Pages 12414-12419

  Talsness, Dana M ^a   Belanto, Joseph J ^a   Ervasti, James M ^a  

^a UNIVERSITY OF MINNESOTA   (United States)

Author keywords

Duchenne muscular dystrophy; Heat shock activator; Missense mutation; Proteasome inhibitor; Protein folding

Indexed keywords

DYSTROPHIN; GEDUNIN; HEAT SHOCK PROTEIN; LIMONOID; PROTEASOME; PROTEASOME INHIBITOR;

AMINO ACID SUBSTITUTION; ARTICLE; CELL DIFFERENTIATION; CONTROLLED STUDY; DOSE RESPONSE; DUCHENNE MUSCULAR DYSTROPHY; EXON SKIPPING; FLOW CYTOMETRY; FLUOROMETRY; IN VITRO STUDY; MISSENSE MUTATION; MYOBLAST; NONHUMAN; PHENOTYPE; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN DEGRADATION; PROTEIN STABILITY; PROTEIN TERTIARY STRUCTURE; PROTEIN UNFOLDING; STEADY STATE; UPREGULATION; ANIMAL; CELL LINE; DRUG EFFECTS; GENETICS; HUMAN; METABOLISM; PATHOLOGY; REVERSE TRANSCRIPTION POLYMERASE CHAIN REACTION; SKELETAL MUSCLE CELL; WESTERN BLOTTING;

ANIMALS; BLOTTING, WESTERN; CELL LINE; DYSTROPHIN; HEAT-SHOCK PROTEINS; HUMANS; LIMONINS; MUSCLE FIBERS, SKELETAL; MUSCULAR DYSTROPHY, DUCHENNE; MUTATION, MISSENSE; PHENOTYPE; PROTEASOME ENDOPEPTIDASE COMPLEX; PROTEASOME INHIBITORS; PROTEIN STABILITY; PROTEOLYSIS; REVERSE TRANSCRIPTASE POLYMERASE CHAIN REACTION;

EID: 84943422476     PISSN: 00278424     EISSN: 10916490     Source Type: Journal    
DOI: 10.1073/pnas.1508755112     Document Type: Article

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