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Article

Wild-type and pathogenic forms of ubiquilin 2 differentially modulate components of the autophagy-lysosome pathways.

Journal of pharmacological sciences - 1 Jul 2023

Idera Akiko, Sharkey Lisa M, Kurauchi Yuki, Kadoyama Keiichi, Paulson Henry L, Katsuki Hiroshi, Seki Takahiro

Abstract excerpt

Missense mutations of ubiquilin 2 (UBQLN2) have been identified to cause X-linked amyotrophic lateral sclerosis (ALS). Proteasome-mediated protein degradation is reported to be impaired by ALS-associated mutations of UBQLN2. However, it remains unknown how these mutations affect autophagy-lysosome protein degradation, which consists of macroautophagy (MA), microautophagy (mA), and chaperone-mediated autophagy...

Topics

Humans
Amyotrophic Lateral Sclerosis
Adaptor Proteins, Signal Transducing
Autophagy
Mutation
Autophagy-Related Proteins
Transcription Factors
Lysosomes

Source

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Identifiers

PMID: 37257946
DOI: 10.1016/j.jphs.2023.05.002
PMCID: PMC12950104

Genes

UBQLN2
LAMP1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 31 Aug 2026.

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Wild-type and pathogenic forms of ubiquilin 2 differentially modulate components of the autophagy-lysosome pathways.PMID 37257946Read ↗

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