Mutant PFN1 causes ALS phenotypes and progressive motor neuron degeneration in mice by a gain of toxicity

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

Volumn 113, Issue 41, 2016, Pages E6209-E6218

  Yang, Chunxing ^a   Danielson, Eric W ^b   Qiao, Tao ^a   Metterville, Jake ^b   Brown, Robert H ^b   Landers, John E ^b   Xu, Zuoshang ^a,b  

^a UNIVERSITY OF MASSACHUSETTS MEDICAL SCHOOL   (United States)

^b UNIVERSITY OF MASSACHUSETTS   (United States)

Author keywords

Denervation; Motor neuron disease; Muscle atrophy; Neurodegeneration; Proteostasis

Indexed keywords

FUSED IN SARCOMA PROTEIN; PROFILIN; PROFILIN 1; PROTEIN P62; TAR DNA BINDING PROTEIN; UBIQUITIN; UNCLASSIFIED DRUG;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; ASTROCYTE; ASTROCYTOSIS; CELL LOSS; CELL STRUCTURE; CONTROLLED STUDY; CYTOPLASM; CYTOSKELETON; DISEASE COURSE; DORSAL ROOT; GLIOSIS; GRIP STRENGTH; HOMOZYGOTE; MICROGLIA; MOTONEURON; MOUSE; MUSCLE WEAKNESS; NERVE CELL; NERVE CELL DEGENERATION; NERVE DEGENERATION; NERVE FIBER DEGENERATION; NERVOUS SYSTEM INFLAMMATION; NEUROFILAMENT; NONHUMAN; PARALYSIS; PHENOTYPE; PRIORITY JOURNAL; PROMOTER REGION; PROTEIN AGGREGATION; PROTEIN EXPRESSION; SPINAL CORD NERVE CELL; TRANSGENIC MOUSE; VENTRAL ROOT; ANIMAL; ANIMAL BEHAVIOR; DISEASE EXACERBATION; DISEASE MODEL; GENE DOSAGE; GENE EXPRESSION; GENETIC ASSOCIATION STUDY; GENETIC PREDISPOSITION; GENETICS; HUMAN; IMMUNOHISTOCHEMISTRY; METABOLISM; MUSCLE ATROPHY; MUTATION; PATHOLOGY; PATHOPHYSIOLOGY; PROTEINOSIS;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMALS; BEHAVIOR, ANIMAL; CYTOSKELETON; DISEASE MODELS, ANIMAL; DISEASE PROGRESSION; GENE DOSAGE; GENE EXPRESSION; GENETIC ASSOCIATION STUDIES; GENETIC PREDISPOSITION TO DISEASE; HUMANS; IMMUNOHISTOCHEMISTRY; MICE; MICE, TRANSGENIC; MOTOR NEURONS; MUSCULAR ATROPHY; MUTATION; NERVE DEGENERATION; PARALYSIS; PHENOTYPE; PROFILINS; PROTEIN AGGREGATION, PATHOLOGICAL;

EID: 84991510961     PISSN: 00278424     EISSN: 10916490     Source Type: Journal    
DOI: 10.1073/pnas.1605964113     Document Type: Article

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