Proteins/UNIPROT:Q6P1S2

MISO1

ProteinUNIPROT:Q6P1S2

Regulates mitochondrial morphology and mitochondrial DNA (mtDNA) homeostasis. Promotes the formation of specialized mitochondrial subdomains called small MTFP1-enriched mitochondria (SMEM). Negatively regulates mitochondrial fusion by excluding fusion coordinator OPA1 from SMEM. Recruits mtDNA, potentially through interaction with SSBP1, and facilitates mtDNA transport to the mitochondrial periphery. Also recruits other proteins, including PHB1, PHB2 and MTFP1, into SMEM which promotes peripheral mitochondrial fission, facilitating lysosome-mediated clearance of damaged mtDNA via mitophagy..

[Isoform 2]: Secreted protein which may play a role in transcription regulation via the MAPK3/MAPK1 pathway through an unidentified receptor on the plasma membrane..

Function from UniProtKB (Q6P1S2), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

Statements

8 statements

8 statements · 2 reference databases · Subject: MISO1 · Sources: gene ontology, Hetionet

Order

MISO1 covaries with GLI1ProteinHetionet hetionet:covaries:2735-285315Inferred only
OR1D5 covaries with MISO1ProteinHetionet hetionet:covaries:285315-8386Inferred only
MISO1 covaries with PHF23ProteinHetionet hetionet:covaries:285315-79142Inferred only
ZNF184 covaries with MISO1ProteinHetionet hetionet:covaries:285315-7738Inferred only
APP interacts with MISO1ProteinHetionet hetionet:interacts:285315-351Inferred only
MISO1 regulates negative regulation of ERK1 and ERK2 cascadeBiological processgene ontology Q6P1S2|GO:0070373Inferred only
MISO1 regulates negative regulation of mitochondrial fusionBiological processgene ontology Q6P1S2|GO:0010637Inferred only
MISO1 regulates positive regulation of mitochondrial fissionBiological processgene ontology Q6P1S2|GO:0090141Inferred only

Other names

5 names

Resolves to: MISO1

Also called: AC3-33C3orf33MISOMitochondrial inner membrane subdomain organizer 1Protein AC3-33

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