Article

MSTO1 functions as a TRiC assembly factor linking cytosolic proteostasis to mitochondrial function

2026-07-22

Abstract excerpt

Bi-allelic mutations in MSTO1 are linked to clinical disease phenotypes characteristic of mitochondrial dysfunction, including ataxia and muscular dystrophy. Consistent with this, MSTO1 patient-derived fibroblasts have fragmented mitochondria and a striking loss of mtDNA. Although MSTO1 has been implicated in regulating mitochondrial fusion, the molecular function of this cytosolic protein in vertebrate cells rem...

Topics

Open a Topic to create a Post that cites this publication.

Identifiers and source

Literature Corpus work: d7d1c09a-43c3-5871-b596-e0d1571342da
DOI: 10.64898/2026.07.21.739937

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

MSTO1 functions as a TRiC assembly factor linking cytosolic proteostasis to mitochondrial functionDOI 10.64898/2026.07.21.739937Read

MSTO1 is a cytoplasmic pro-mitochondrial fusion protein, whose mutation induces myopathy and ataxia in humans.Semantic proximity Read

Recessive mutations in MSTO1 cause mitochondrial dynamics impairment, leading to myopathy and ataxia.Semantic proximity Read

When mitochondria fall apart: Unbalanced mitochondrial segregation triggers loss of mtDNA in the absence of mitochondrial fusionSemantic proximity Read

Endoplasmic reticulum-associated mitochondria-cortex tether functions in the distribution and inheritance of mitochondria.Semantic proximity Read

Tetracycline-dependent inhibition of mitoribosome protein elongation in mitochondrial disease mutant cells suppresses IRE1α to promote cell survivalSemantic proximity Read

The Mitochondrial F-box protein 1 and DJ-1 homolog HSP31 support cellular proteostasis during mitochondrial protein import cloggingSemantic proximity Read

Wounding triggers MIRO-1 dependent mitochondrial fragmentation that accelerates epidermal wound closure through oxidative signalingSemantic proximity Read

Mitochondrial nucleoid organization and biogenesis of complex I require mTERF18/SHOT1 and ATAD3 in <i>Arabidopsis thaliana</i>Semantic proximity Read

Mitochondrial fission and fusion mediators, hFis1 and OPA1, modulate cellular senescence.Semantic proximity Read

LONP1 is required for maturation of a subset of mitochondrial proteins and its loss elicits an integrated stress responseSemantic proximity Read

A novel homozygous MSTO1 mutation in Ashkenazi Jewish siblings with ataxia and myopathy.Semantic proximity Read

Tau promotes neurodegeneration via DRP1 mislocalization in vivo.Semantic proximity Read