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.

单词	amyotrophic lateral sclerosis type 14
释义	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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