Caudal-Rostral Progression of Alpha Motoneuron Degeneration in the SOD1^G93A Mouse Model of Amyotrophic Lateral Sclerosis.

ABSTRACT: Mice with transgenic expression of human SOD1^G93A are a widely used model of ALS, with a caudal-rostral progression of motor impairment. Previous studies have quantified the progression of motoneuron (MN) degeneration based on size, even though alpha (α-) and gamma (γ-) MNs overlap in size. Therefore, using molecular markers and synaptic inputs, we quantified the survival of α-MNs and γ-MNs at the lumbar and cervical spinal segments of 3- and 4-month SOD1^G93A mice, to investigate whether there is a caudal-rostral progression of MN death. By 3 months, in the cervical and lumbar spinal cord, there was α-MN degeneration with complete γ-MN sparing. At 3 months, the cervical spinal cord had more α-MNs per ventral horn than the lumbar spinal cord in SOD1^G93A mice

SUBMITTER: Kirby AJ 

PROVIDER: S-EPMC9137889 | biostudies-literature | 2022 May

REPOSITORIES: biostudies-literature