Respiratory pathology in the Optn^-/- mouse model of Amyotrophic Lateral Sclerosis.

ABSTRACT: Amyotrophic Lateral Sclerosis (ALS) is a devastating neurodegenerative disorder that results in death due to respiratory failure. Many genetic defects are associated with ALS; one such defect is a mutation in the gene encoding optineurin (OPTN). Using an optineurin null mouse (Optn^-/-), we sought to characterize the impact of optineurin deficiency on respiratory neurodegeneration. Respiratory function was assessed at 6 and 12 mo of age using whole body plethysmography at baseline during normoxia (FiO₂: 0.21; N₂ balance) and during a respiratory challenge with hypoxia and hypercapnia (FiCO₂: 0.07, FiO₂: 0.10; N₂ balance). Histological analyses to assess motor neuron viability and respiratory nerve integrity were performed in

SUBMITTER: McCall AL 

PROVIDER: S-EPMC7530116 | biostudies-literature | 2020 Nov

REPOSITORIES: biostudies-literature