Differences in protein quality control correlate with phenotype variability in 2 mouse models of familial amyotrophic lateral sclerosis

Neurobiology of Aging

Volumn 36, Issue 1, 2015, Pages 492-504

  Marino, Marianna ^a   Papa, Simonetta ^a   Crippa, Valeria ^b   Nardo, Giovanni ^a   Peviani, Marco ^a,d   Cheroni, Cristina ^a,e   Trolese, Maria Chiara ^a   Lauranzano, Eliana ^a   Bonetto, Valentina ^a   Poletti, Angelo ^b   DeBiasi, Silvia ^b   Ferraiuolo, Laura ^c,f   Shaw, Pamela J ^c   Bendotti, Caterina ^a  

^a MARIO NEGRI INSTITUTE FOR PHARMACOLOGICAL RESEARCH   (Italy)

^b UNIVERSITY OF MILAN   (Italy)

^c UNIVERSITY OF SHEFFIELD   (United Kingdom)

^d UNIVERSITY OF PAVIA   (Italy)

^e Istituto Nazionale di Genetica Molecolare   (Italy)

^f THE RESEARCH INSTITUTE AT NATIONWIDE CHILDREN S HOSPITAL   (United States)

Author keywords

Alpha B crystallin; ALS; Autophagy; Chaperone; Cyclophillin A; Proteasome; Protein quality control; SOD1G93A transgenic mouse

Indexed keywords

ALPHA CRYSTALLIN; COPPER ZINC SUPEROXIDE DISMUTASE; CYCLOPHILIN A; HEAT SHOCK PROTEIN 70; HEAT SHOCK PROTEIN 90; POLYUBIQUITIN; PROTEASOME; TAR DNA BINDING PROTEIN; CHAPERONE; DNA BINDING PROTEIN; MESSENGER RNA; SOD1 G93A PROTEIN; SUPEROXIDE DISMUTASE; TDP-43 PROTEIN, MOUSE;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; AUTOPHAGY LYSOSOMAL PATHWAY; BIOCHEMISTRY; CELL LOSS; COMPARATIVE STUDY; CONTROLLED STUDY; CORRELATION ANALYSIS; DISEASE SEVERITY; DOWN REGULATION; FAMILIAL AMYOTROPHIC LATERAL SCLEROSIS; FAMILIAL DISEASE; FEMALE; HISTOPATHOLOGY; MOLECULAR MECHANICS; MOTONEURON; MOUSE; NONHUMAN; PHENOTYPIC VARIATION; PROTEIN AGGREGATION; PROTEIN DEGRADATION; PROTEIN EXPRESSION; PROTEIN LOCALIZATION; PROTEIN PROCESSING; PROTEIN QUALITY CONTROL; UBIQUITIN PROTEASOME SYSTEM; ANIMAL; CENTRAL NERVOUS SYSTEM; DISEASE COURSE; DISEASE MODEL; GENETICS; METABOLISM; MOLECULARLY TARGETED THERAPY; PATHOLOGY; PHENOTYPE; PROTEINOSIS; SEVERITY OF ILLNESS INDEX; TRANSGENIC MOUSE;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMALS; CENTRAL NERVOUS SYSTEM; CYCLOPHILIN A; DISEASE MODELS, ANIMAL; DISEASE PROGRESSION; DNA-BINDING PROTEINS; MICE, TRANSGENIC; MOLECULAR CHAPERONES; MOLECULAR TARGETED THERAPY; MOTOR NEURONS; PHENOTYPE; PROTEASOME ENDOPEPTIDASE COMPLEX; PROTEIN AGGREGATION, PATHOLOGICAL; RNA, MESSENGER; SEVERITY OF ILLNESS INDEX; SUPEROXIDE DISMUTASE;

EID: 84920609049     PISSN: 01974580     EISSN: 15581497     Source Type: Journal    
DOI: 10.1016/j.neurobiolaging.2014.06.026     Document Type: Article

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