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Article

Loss of TRPML1 promotes production of reactive oxygen species: is oxidative damage a factor in mucolipidosis type IV?

The Biochemical journal - 15 Jan 2014

Coblentz Jessica, St Croix Claudette, Kiselyov Kirill

Abstract excerpt

TRPML1 (transient receptor potential mucolipin 1) is a lysosomal ion channel permeable to cations, including Fe2+. Mutations in MCOLN1, the gene coding for TRPML1, cause the LSD (lysosomal storage disease) MLIV (mucolipidosis type IV). The role of TRPML1 in the cell is disputed and the mechanisms of cell deterioration in MLIV are unclear. The demonstration of Fe2+ buildup in MLIV cells raised the possibility that...

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Source

Identifiers

PMID: 24192042
DOI: 10.1042/BJ20130647

Genes

MCOLN1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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