MISO1
entrez
285315
canonical
uniprot
Q6P1S2
canonical
ensembl
ENSG00000174928
resolver
ensp
ENSP00000342512
resolver
ensp
ENSP00000445446
resolver
ensp
ENSP00000531478
resolver
genesymbol
MISO1
resolver
genesymbol_syn
C3orf33
resolver
genesymbol_syn
MISO
resolver
hgnc
HGNC:26434
resolver
uniprot
A8K1H5
resolver
uniprot
Q86YE6
resolver
uniprot
Q8IXA7
resolver
uniprot
Q96NB5
resolver
uniprot_entry
MISO1_HUMAN
resolver
uniprot_sec
A8K1H5
resolver
uniprot_sec
Q86YE6
resolver
uniprot_sec
Q8IXA7
resolver
uniprot_sec
Q96NB5
resolver
amino_acid_sequence
MAGQPAATGSPSADKDGMEPNVVARISQWADDHLRLVRNISTGMAIAGIMLLLRSIRLTSKFTSSSDIPVEFIRRNVKLRGRLRRITENGLEIEHIPITLPIIASLRKEPRGALLVKLAGVELAETGKAWLQKELKPSQLLWFQLLGKENSALFCYLLVSKGGYFSVNLNEEILRRGLGKTVLVKGLKYDSKIYWTVHRNLLKAELTALKKGEGIWKEDSEKESYLEKFKDSWREIWKKDSFLKTTGSDFSLKKESYYEKLKRTYEIWKDNMNNCSLILKFRELISRINFRRKG
uniprot
proteins
function
FUNCTION: Regulates mitochondrial morphology and mitochondrial DNA (mtDNA) homeostasis (PubMed:41398051). Promotes the formation of specialized mitochondrial subdomains called small MTFP1-enriched mitochondria (SMEM) (PubMed:41398051). Negatively regulates mitochondrial fusion by excluding fusion coordinator OPA1 from SMEM (PubMed:41398051). Recruits mtDNA, potentially through interaction with SSBP1, and facilitates mtDNA transport to the mitochondrial periphery (PubMed:41398051). 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 (PubMed:41398051). {ECO:0000269|PubMed:41398051}.; FUNCTION: [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. {ECO:0000269|PubMed:20680465}.
uniprot
proteins
mass_dalton
33765
uniprot
proteins
post_translational_modification
PTM: Rapidly degraded under basal conditions which maintains low steady-state levels of the protein within mitochondria (PubMed:41398051). Degradation is inhibited by inner mitochondrial membrane stresses including mtDNA damage, OXPHOS dysfunction and cristae disruption, leading to accumulation of the protein under stress conditions (PubMed:41398051). {ECO:0000269|PubMed:41398051}.
uniprot
proteins
protein_family
MISO1 family
uniprot
proteins
pubmed
14702039
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
16641997
uniprot
proteins
pubmed
19413330
uniprot
proteins
pubmed
20680465
uniprot
proteins
pubmed
41398051
uniprot
proteins
sequence_length
294
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: [Isoform 1]: Mitochondrion inner membrane {ECO:0000269|PubMed:41398051}; Single-pass membrane protein {ECO:0000255}. Note=Assembles into discrete subdomains known as small MTFP1-enriched mitochondria (SMEM). {ECO:0000269|PubMed:41398051}.; SUBCELLULAR LOCATION: [Isoform 2]: Secreted {ECO:0000269|PubMed:20680465}.
uniprot
proteins
transmembrane_region
TRANSMEM 40..56; /note="Helical"; /evidence="ECO:0000255
uniprot
proteins