Cyclin F, Neurodegeneration, and the Pathogenesis of ALS/FTD

Amyotrophic lateral sclerosis (ALS) is the most common form of motor neuron disease and is characterized by the degeneration of upper and lower motor neurons of the brain and spinal cord. ALS is also linked clinically, genetically, and pathologically to a form of dementia known as frontotemporal dem...

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Veröffentlicht in:The Neuroscientist 2024-04, Vol.30 (2), p.214-228
Hauptverfasser: Rayner, Stephanie L., Hogan, Alison, Davidson, Jennilee M., Cheng, Flora, Luu, Luan, Morsch, Marco, Blair, Ian, Chung, Roger, Lee, Albert
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container_end_page 228
container_issue 2
container_start_page 214
container_title The Neuroscientist
container_volume 30
creator Rayner, Stephanie L.
Hogan, Alison
Davidson, Jennilee M.
Cheng, Flora
Luu, Luan
Morsch, Marco
Blair, Ian
Chung, Roger
Lee, Albert
description Amyotrophic lateral sclerosis (ALS) is the most common form of motor neuron disease and is characterized by the degeneration of upper and lower motor neurons of the brain and spinal cord. ALS is also linked clinically, genetically, and pathologically to a form of dementia known as frontotemporal dementia (FTD). Identifying gene mutations that cause ALS/FTD has provided valuable insight into the disease process. Several ALS/FTD-causing mutations occur within proteins with roles in protein clearance systems. This includes ALS/FTD mutations in CCNF, which encodes the protein cyclin F: a component of a multiprotein E3 ubiquitin ligase that mediates the ubiquitylation of substrates for their timely degradation. In this review, we provide an update on the link between ALS/FTD CCNF mutations and neurodegeneration.
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subjects Amyotrophic Lateral Sclerosis - genetics
Amyotrophic Lateral Sclerosis - metabolism
Cyclins - genetics
Cyclins - metabolism
Frontotemporal Dementia - genetics
Frontotemporal Dementia - metabolism
Humans
Motor Neurons - metabolism
Mutation
title Cyclin F, Neurodegeneration, and the Pathogenesis of ALS/FTD
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