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Article

Loss of TMEM106B exacerbates C9ALS/FTD DPR pathology by disrupting autophagosome maturation

16 Dec 2022

Abstract excerpt

Disruption to protein homeostasis caused by lysosomal dysfunction and associated impairment of autophagy is a prominent pathology in amyotrophic lateral sclerosis and frontotemporal dementia (ALS/FTD). The most common genetic cause of ALS/FTD is a G4C2 hexanucleotide repeat expansion in C9orf72 (C9ALS/FTD). Repeat-associated non-AUG (RAN) translation of G4C2 repeat transcripts gives rise to dipeptide repeat (DPR)...

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Identifiers

PMID: 36619668
DOI: 10.3389/fncel.2022.1061559

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Loss of TMEM106B exacerbates C9ALS/FTD DPR pathology by disrupting autophagosome maturationPMID 36619668Read ↗

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Loss of TMEM106B Ameliorates Lysosomal and Frontotemporal Dementia-Related Phenotypes in Progranulin-Deficient Mice.Semantic proximity Read ↗

TMEM106B, a frontotemporal lobar dementia (FTLD) modifier, associates with FTD-3-linked CHMP2B, a complex of ESCRT-III.Semantic proximity Read ↗

The frontotemporal lobar degeneration risk factor, TMEM106B, regulates lysosomal morphology and functionSemantic proximity Read ↗

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VCP modulation ameliorates pathological features in C9orf72 models.Semantic proximity Read ↗

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