amyotrophic lateral sclerosis type 8

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amyotrophic lateral sclerosis type 8

A hereditary neurodegenerative disorder (OMIM:608627) which affects upper motor neurons in the brain and lower motor neurons in the brain stem and spinal cord, resulting in fatal paralysis; sensory abnormalities are absent. Death usually occurs within 2 to 5 years.

Molecular pathology
Defects of VAPB, which encodes a membrane protein involved in vesicle trafficking, cause amyotrophic lateral sclerosis type 8.
References in periodicals archive ?
Downregulation of VAPB expression in motor neurons derived from induced pluripotent stem cells of ALS8 patients.