The proline/arginine dipeptide from hexanucleotide repeat expanded C9ORF72 inhibits the proteasome

eNeuro

Volumn 4, Issue 1, 2017, Pages

  Gupta, Rahul ^a,b   Lan, Matthews ^b   Mojsilovic Petrovic, Jelena ^c   Choi, Won Hoon ^d   Safren, Nathaniel ^e   Barmada, Sami ^e   Lee, Min Jae ^d   Kalb, Robert ^c,f  

^a UNIVERSITY OF PENNSYLVANIA   (United States)

^b UNIVERSITY OF PENNSYLVANIA   (United States)

^c CHILDREN S HOSPITAL OF PHILADELPHIA   (United States)

^d Seoul National University College of Medicine   (South Korea)

^e UNIVERSITY OF MICHIGAN MEDICAL SCHOOL   (United States)

^f UNIVERSITY OF PENNSYLVANIA   (United States)

Author keywords

ALS; Frontotemporal dementia; Lysosome autophagy; Motor neuron; Proteasome

Indexed keywords

ARGININE; BAFILOMYCIN A1; BENZYLOXYCARBONYLLEUCYLLEUCYLLEUCINAL; DIPEPTIDE; MAMMALIAN TARGET OF RAPAMYCIN; PEPTIDES AND PROTEINS; PROLINE; PROLINE ARGININE DIPEPTIDE PR20; PROTEASOME; REPEAT ASSOCIATED NON ATG PEPTIDE; SUPEROXIDE DISMUTASE; TAR DNA BINDING PROTEIN; UNCLASSIFIED DRUG; GR20 PEPTIDE; GUANINE NUCLEOTIDE EXCHANGE FACTOR; PEPTIDE; PR20 PEPTIDE; PROTEASOME INHIBITOR; REPETITIVE DNA;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMAL CELL; ARTICLE; ASTROCYTE CULTURE; AUTOPHAGOSOME; BIOCHEMICAL ANALYSIS; C9ORF72 GENE; CELL INTERACTION; CONTROLLED STUDY; CYTOLYSIS ASSAY; CYTOTOXICITY ASSAY; ENDOPLASMIC RETICULUM STRESS; FLUORESCENCE MICROSCOPY; FLUX ASSAY; FRONTOTEMPORAL DEMENTIA; GENE; GENE MUTATION; HUMAN; HUMAN CELL; IMMUNOCYTOCHEMISTRY; IMMUNOREACTIVITY; MITOPHAGY; MOTONEURON; NEWBORN; NONHUMAN; POLYACRYLAMIDE GEL ELECTROPHORESIS; PRIORITY JOURNAL; RAT; SPINAL CORD CELL CULTURE; UBIQUITINATION; WESTERN BLOTTING; XTT ASSAY; ANIMAL; ASTROCYTE; BRAIN CORTEX; CELL CULTURE; CELL DEATH; CHEMISTRY; COCULTURE; DRUG EFFECTS; GENETICS; HEK293 CELL LINE; METABOLISM; NERVE CELL; PATHOLOGY; PHYSIOLOGY; SPINAL CORD; SPRAGUE DAWLEY RAT;

ANIMALS; ASTROCYTES; CELL DEATH; CELLS, CULTURED; CEREBRAL CORTEX; COCULTURE TECHNIQUES; DNA REPEAT EXPANSION; GUANINE NUCLEOTIDE EXCHANGE FACTORS; HEK293 CELLS; HUMANS; NEURONS; PEPTIDES; PROTEASOME ENDOPEPTIDASE COMPLEX; PROTEASOME INHIBITORS; RATS, SPRAGUE-DAWLEY; SPINAL CORD;

EID: 85021337721     PISSN: None     EISSN: 23732822     Source Type: Journal    
DOI: 10.1523/ENEURO.0249-16.2017     Document Type: Article

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