ALS-associated mutation SOD1G93A leads to abnormal mitochondrial dynamics in osteocytes.

ABSTRACT: While the death of motor neuron is a pathological hallmark of amyotrophic lateral sclerosis (ALS), defects in other cell types or organs may also actively contribute to ALS disease progression. ALS patients experience progressive skeletal muscle wasting that may not only exacerbate neuronal degeneration, but likely has a significant impact on bone function. In our previous published study, we have discovered severe bone loss in an ALS mouse model with overexpression of ALS-associated mutation SOD1G93A (G93A). Here we further provide a mechanistic understanding of the bone loss in ALS animal and cellular models. Combining mitochondrial fluorescent indicators and confocal live cell imaging, we discovered abnormalities in mitochondrial network and dynamics in primary osteocytes derived from t

SUBMITTER: Wang H 

PROVIDER: S-EPMC5718158 | biostudies-literature | 2018 Jan

REPOSITORIES: biostudies-literature