Publications by authors named "Evelyn R Scarrow"
Article Synopsis
- Amyotrophic lateral sclerosis (ALS) is a severe neurodegenerative disease leading to death within 2-5 years, with respiratory failure being the most common cause.
- This study focused on TDP-43 mutations in a Prp-hTDP-43 mouse model to investigate their impact on respiratory motor units, revealing significant breathing deficits and neuroinflammation.
- The findings indicate that TDP-43 mice exhibit marked respiratory and neuropathological issues, making them a valuable model for researching potential therapies for ALS-related breathing problems.
Spinocerebellar ataxia type 7 (SCA7) is an autosomal dominant neurological disorder caused by deleterious CAG repeat expansion in the coding region of the ataxin 7 gene (polyQ-ataxin-7). Infantile-onset SCA7 leads to severe clinical manifestation of respiratory distress, but the exact cause of respiratory impairment remains unclear. Using the infantile-SCA7 mouse model, the SCA7266Q/5Q mouse, we examined the impact of pathological polyQ-ataxin-7 on hypoglossal (XII) and phrenic motor units.
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