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Misato mitochondrial distribution and morphology regulator 1 (MSTO1)
- Target
- MSTO1
- Molecular classification
- Other (cytoplasmic protein involved in organelle dynamics, not a classical receptor, enzyme, transporter, etc.)
01
Overview
Misato mitochondrial distribution and morphology regulator 1 (MSTO1) is an evolutionarily conserved, nuclear-encoded protein primarily localized in the cytoplasm with partial association at the mitochondrial outer membrane[1][2][4]. It shows homology to the tubulin/FtsZ GTPase superfamily and is essential for promoting mitochondrial fusion and maintaining the continuity and morphology of the mitochondrial network; loss of MSTO1 function leads to mitochondrial fragmentation, aggregation, and reduced mitochondrial DNA content without directly altering core bioenergetic enzymes[1][2][4]. Clinically, MSTO1 mutations are linked to autosomal dominant and recessive diseases presenting as mitochondrial myopathy, multisystem involvement, or neurodegenerative features; MSTO1 is not currently a therapeutic target and no drug interactions or targeted therapies are known[1][2][4][5].
- Other names
- Protein misato homolog 1LST005SLTP005FLJ10504MSTmisatoMMYATmisato 1misato homolog 1mitochondrial distribution and morphology regulator
02
Biological functions
Mitochondrial fusionRegulation of mitochondrial morphologyRegulation of mitochondrial distributionMaintenance of mitochondrial DNA integrityCell division and chromosome segregation
03
Disease associations
Neurodegenerative diseaseMitochondrial myopathyMultisystem disease with cerebellar atrophy and ataxia
04
Safety considerations
No specific therapeutic safety issues detailed, but loss-of-function mutations lead to mitochondrial dysfunction and may be relevant for neurodegenerative/myopathic disease phenotype[1][4].
05
Biomarkers
MSTO1 protein or mRNA abundance (for mitochondrial fusion defects)
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