Ontology highlight
ABSTRACT:
SUBMITTER: Cortes CJ
PROVIDER: S-EPMC4180729 | biostudies-literature | 2014 Sep
REPOSITORIES: biostudies-literature
Cortes Constanza J CJ Miranda Helen C HC Frankowski Harald H Batlevi Yakup Y Young Jessica E JE Le Amy A Ivanov Nishi N Sopher Bryce L BL Carromeu Cassiano C Muotri Alysson R AR Garden Gwenn A GA La Spada Albert R AR
Nature neuroscience 20140810 9
Macroautophagy (hereafter autophagy) is a key pathway in neurodegeneration. Despite protective actions, autophagy may contribute to neuron demise when dysregulated. Here we consider X-linked spinal and bulbar muscular atrophy (SBMA), a repeat disorder caused by polyglutamine-expanded androgen receptor (polyQ-AR). We found that polyQ-AR reduced long-term protein turnover and impaired autophagic flux in motor neuron-like cells. Ultrastructural analysis of SBMA mice revealed a block in autophagy pa ...[more]