Increased levels of UCHL1 are a compensatory response to disrupted ubiquitin homeostasis in spinal muscular atrophy and do not represent a viable therapeutic target

Neuropathology and Applied Neurobiology

Volumn 40, Issue 7, 2014, Pages 873-887

  Powis, Rachael A ^a   Mutsaers, Chantal A ^a   Wishart, Thomas M ^a,b   Hunter, Gillian ^a   Wirth, Brunhilde ^c   Gillingwater, Thomas H ^a  

^a UNIVERSITY OF EDINBURGH   (United Kingdom)

^b UNIVERSITY OF EDINBURGH   (United Kingdom)

^c UNIVERSITY OF COLOGNE   (Germany)

Author keywords

SMA; Spinal muscular atrophy; Uba1; Ubiquitin; UCHL1

Indexed keywords

INDOLE DERIVATIVE; LDN 57444; OXIME; PGP9.5 PROTEIN, MOUSE; SMN1 PROTEIN, MOUSE; SURVIVAL MOTOR NEURON PROTEIN 1; UBIQUITIN THIOLESTERASE;

ANIMAL; ANTAGONISTS AND INHIBITORS; CELL CULTURE; DISEASE MODEL; DRUG EFFECTS; ENZYMOLOGY; FIBROBLAST; HOMEOSTASIS; HUMAN; METABOLISM; MOTONEURON; MOTOR ACTIVITY; MOUSE; MUSCULAR ATROPHY, SPINAL; PATHOLOGY;

ANIMALS; CELLS, CULTURED; DISEASE MODELS, ANIMAL; FIBROBLASTS; HOMEOSTASIS; HUMANS; INDOLES; MICE; MOTOR ACTIVITY; MOTOR NEURONS; MUSCULAR ATROPHY, SPINAL; OXIMES; SURVIVAL OF MOTOR NEURON 1 PROTEIN; UBIQUITIN THIOLESTERASE;

EID: 84908887829     PISSN: 03051846     EISSN: 13652990     Source Type: Journal    
DOI: 10.1111/nan.12168     Document Type: Article

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