Ontology highlight
ABSTRACT:
SUBMITTER: Xie Y
PROVIDER: S-EPMC4511489 | biostudies-literature | 2015 Jul
REPOSITORIES: biostudies-literature
Xie Yuxiang Y Zhou Bing B Lin Mei-Yao MY Wang Shiwei S Foust Kevin D KD Sheng Zu-Hang ZH
Neuron 20150701 2
One pathological hallmark in ALS motor neurons (MNs) is axonal accumulation of damaged mitochondria. A fundamental question remains: does reduced degradation of those mitochondria by an impaired autophagy-lysosomal system contribute to mitochondrial pathology? We reveal MN-targeted progressive lysosomal deficits accompanied by impaired autophagic degradation beginning at asymptomatic stages in fALS-linked hSOD1(G93A) mice. Lysosomal deficits result in accumulation of autophagic vacuoles engulfin ...[more]