Miso1
entrez
329659
canonical
uniprot
Q8BN57
canonical
ensembl
ENSMUSG00000048581
resolver
ensp
ENSMUSP00000058114
resolver
enst
ENSMUST00000061706
resolver
genesymbol
Miso1
resolver
uniprot
Q059W9
resolver
uniprot_entry
MISO1_MOUSE
resolver
uniprot_sec
Q059W9
resolver
amino_acid_sequence
MARPPASLGSQAPDRDRGEANVVTRVSQWADNHLRLVQNISTGMAIAGIMLLIRSVRLTSKFTTSSDIPVEFIRKKVKLRGRLQRITECGLEIEHIPITLPFISSWKEEPRGVLLVKLAGVELTESGKVWLQAELKPSQLLWFQLLGKEDSALFCYLLVNKGGYFNVNLNEEILRRGLGKTVLVKGLNYDSKTHWKIHRNLLKAELTALKKGEGIWKEESEKESYFRKLKDSWRERWTKDNDLKPAGADLGSTKDSYHDSRRRASGKGKDSVSNYSFFLKLREFVSRLHFWRKG
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) (By similarity). Negatively regulates mitochondrial fusion by excluding fusion coordinator OPA1 from SMEM (By similarity). Recruits mtDNA, potentially through interaction with SSBP1, and facilitates mtDNA transport to the mitochondrial periphery (By similarity). 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 (By similarity). {ECO:0000250|UniProtKB:Q6P1S2, ECO:0000269|PubMed:41398051}.
uniprot
proteins
mass_dalton
33668
uniprot
proteins
post_translational_modification
PTM: Rapidly degraded under basal conditions which maintains low steady-state levels of the protein within mitochondria. 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. {ECO:0000250|UniProtKB:Q6P1S2}.
uniprot
proteins
protein_family
MISO1 family
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
16141072
uniprot
proteins
pubmed
41398051
uniprot
proteins
sequence_length
294
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: 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}.
uniprot
proteins
transmembrane_region
TRANSMEM 36..53; /note="Helical"; /evidence="ECO:0000255
uniprot
proteins