Heterogeneity in the properties of NEFL mutants causing Charcot-Marie-Tooth disease results in differential effects on neurofilament assembly and susceptibility to intervention by the chaperone-inducer, celastrol

International Journal of Biochemistry and Cell Biology

Volumn 45, Issue 7, 2013, Pages 1499-1508

  Gentil, Benoit J ^a   Mushynski, Walter E ^b   Durham, Heather D ^a  

^a MCGILL UNIVERSITY   (Canada)

^b MCGILL UNIVERSITY   (Canada)

Author keywords

Charcot Marie Tooth disease celastrol; Heat shock proteins; Neurofilament aggregation; Neurofilament light protein

Indexed keywords

CELASTROL; CHAPERONE; DIMER; HEAT SHOCK PROTEIN; HEAT SHOCK PROTEIN 27; MUTANT PROTEIN; NEUROFILAMENT PROTEIN; HEAT SHOCK PROTEIN 70; HSPA1A PROTEIN, RAT; HSPA1B PROTEIN, HUMAN; HSPB1 PROTEIN, HUMAN; NEUROFILAMENT PROTEIN L; TRIPTERINE; TRITERPENE;

ANIMAL CELL; ARTICLE; CELL INCLUSION; CELL SIZE; CONTROLLED STUDY; EMBRYO; GENE MUTATION; HEREDITARY MOTOR SENSORY NEUROPATHY; IN VITRO STUDY; MITOCHONDRIAL VOLUME; MOTONEURON; MOUSE; NERVE CELL CULTURE; NEUROFILAMENT; NEUROPROTECTION; NONHUMAN; PROTEIN ASSEMBLY; PROTEIN DOMAIN; PROTEIN EXPRESSION; PROTEIN FOLDING; PROTEIN POLYMORPHISM; SENSORY NERVE CELL; UPREGULATION; ANIMAL; BIOSYNTHESIS; CHEMISTRY; GENETICS; HUMAN; METABOLISM; MITOCHONDRION; TUMOR CELL LINE;

ANIMALS; CELL LINE, TUMOR; CHARCOT-MARIE-TOOTH DISEASE; HSP27 HEAT-SHOCK PROTEINS; HSP70 HEAT-SHOCK PROTEINS; HUMANS; MICE; MITOCHONDRIA; MOTOR NEURONS; NEUROFILAMENT PROTEINS; PROTEIN FOLDING; SENSORY RECEPTOR CELLS; TRITERPENES;

EID: 84878934230     PISSN: 13572725     EISSN: 18785875     Source Type: Journal    
DOI: 10.1016/j.biocel.2013.04.009     Document Type: Article

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