amyotrophic lateral sclerosis type 14

amyotrophic lateral sclerosis type 14

A hereditary neurodegenerative disorder (OMIM:613954) which affects upper motor neurons in the brain and lower motor neurons in the brain stem and spinal cord, resulting in frontotemporal dementia and fatal paralysis; sensory abnormalities are absent.

Pathology
Pallor of the corticospinal tract due to loss of motor neurons, ubiquitin-positive inclusions in surviving motor neurons and deposition of pathologic aggregates.

Molecular pathology
Defects of VCP, which encodes a putative ATP-binding protein involved in vesicle transport and mitotic events, cause amyotrophic lateral sclerosis type 14.
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