Axonal transport of CHMP2b is regulated by kinesin-binding protein and disrupted by CHMP2bintron5
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Summary
It is shown how the frontotemporal dementia–causative CHMP2bintron5 mutation disrupts its transport and synaptic localization, and how this phenotype is caused by deficient binding of CHMP2bintron5 to kinesin-binding protein, which is identified as a key regulator of CHMP2b transport.
- Type
- article
- Published
- 2025-02-28
- Cited by
- 1
- References
- 60
- Access
- Open access
- OpenAlex
- https://openalex.org/W4408049789
- Semantic Scholar
- https://api.semanticscholar.org/CorpusID:276716033
Keywords
Kinesin, Cell biology, ESCRT, Synaptic vesicle, Biology
References
- Dysregulated molecular pathways in amyotrophic lateral sclerosis–frontotemporal dementia spectrum disorder
- Molecular Genetics of Frontotemporal Dementia Elucidated by Drosophila Models—Defects in Endosomal–Lysosomal Pathway
- Rab proteins as membrane organizers
- Frontotemporal dementia caused by CHMP2B mutation is characterised by neuronal lysosomal storage pathology
- Axonal autophagosomes recruit dynein for retrograde transport through fusion with late endosomes
- The ESCRT pathway.
- Progressive neuronal inclusion formation and axonal degeneration in CHMP2B mutant transgenic mice.
- ESCRT-III recognition by VPS4 ATPases
- The ESCRT machinery in endosomal sorting of ubiquitylated membrane proteins
- Loss of hrs in the central nervous system causes accumulation of ubiquitinated proteins and neurodegeneration.
- Synaptic vesicle trafficking and Parkinson's disease
- ESCRT-III dysfunction causes autophagosome accumulation and neurodegeneration.
- Mutations in the endosomal ESCRTIII-complex subunit CHMP2B in frontotemporal dementia
- Ordered assembly of the ESCRT-III complex on endosomes is required to sequester cargo during MVB formation.
- AXONAL TRANSPORT: CARGO-SPECIFIC MECHANISMS OF MOTILITY AND REGULATION
- CHMP2B mutations are rare in French families with frontotemporal lobar degeneration
- The synaptic maintenance problem: membrane recycling, Ca2+ homeostasis and late onset degeneration
- CHMP2B C-truncating mutations in frontotemporal lobar degeneration are associated with an aberrant endosomal phenotype in vitro.
- Lysosomal proteolysis inhibition selectively disrupts axonal transport of degradative organelles and causes an Alzheimer’s-like axonal dystrophy
- ALS phenotypes with mutations in CHMP2B (charged multivesicular body protein 2B)
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