Autonomic nervous system involvement in the giant axonal neuropathy (GAN) KO mouse: implications for human disease.

ABSTRACT:

Purpose

Giant axonal neuropathy (GAN) is an inherited severe sensorimotor neuropathy. The aim of this research was to investigate the neuropathologic features and clinical autonomic nervous system (ANS) phenotype in two GAN knockout (KO) mouse models. Little is known about ANS involvement in GAN in humans, but autonomic signs and symptoms are commonly reported in early childhood.

Methods

Routine histology and immunohistochemistry was performed on GAN KO mouse specimens taken at various ages. Enteric dysfunction was assessed by quantifying the frequency, weight, and water content of defecation in GAN KO mice.

Results

Histological examination of the enteric, parasympathetic and sympathetic ANS of GAN KO mice revealed pronounced and widespread neuronal perikaryal interme

SUBMITTER: Armao D 

PROVIDER: S-EPMC5057391 | biostudies-literature | 2016 Aug

REPOSITORIES: biostudies-literature