Mios
entrez
252875
canonical
uniprot
Q8VE19
canonical
ensembl
ENSMUSG00000042447
resolver
ensp
ENSMUSP00000039301
resolver
ensp
ENSMUSP00000257694
resolver
ensp
ENSMUSP00000257698
resolver
ensp
ENSMUSP00000257703
resolver
enst
ENSMUST00000040017
resolver
genesymbol
Mios
resolver
genesymbol_syn
BC020002
resolver
uniprot
E9QM00
resolver
uniprot
Q8BV62
resolver
uniprot_entry
MIOS_MOUSE
resolver
uniprot_entry
Q8BV62_MOUSE
resolver
uniprot_sec
E9QM00
resolver
activity_regulation
ACTIVITY REGULATION: The GATOR2 complex is negatively regulated by the upstream amino acid sensors CASTOR1 and SESN2, which sequester the GATOR2 complex in absence of amino acids. In the presence of abundant amino acids, GATOR2 is released from CASTOR1 and SESN2 and activated. {ECO:0000250|UniProtKB:Q9NXC5}.
uniprot
proteins
amino_acid_sequence
MSGTKPDILWAPHQVDRFVVCDSELSLYHVESAVNSELKAGSLRLSEDSAATLLSINSDTPYMKCVAWYLNYDPECLLAVGQANGRVVLTSLGQDHNSKFKDLIGKEFVPKHARQCNTLAWNPLDSNWLAAGLDKHRADFSVLIWDICSKYTPDIVPMEKVRLSAGEAETTLLVTKPLYELGQNDASLSLCWLPRDQKLLLAGMHRNLAIFDLRNTSQKMFVNTKAVQGVTVDPYFHDRVASFYEGQVAIWDLRKFEKPVLTLTEQPKPLTKVAWCPTRTGLLATLTRDSNIIRLYDMQHTPTPIGDETEPTIIERSVQPCDNYIASFAWHPTSQNRMIVVTPNRTMSDFTVFERISLAWSPITSLMWACGRHLYECAEEESDNSLEKDIATKMRLRALSRYGLDTEQVWRNHILAGNEDPQLKSLWYTLHFMKQYTEDNDQKSPGNKGSLVYAGIKSIVKSSLGMVESSRHNWSGLDKQTDIQNLNEERILALQLCGWIKKGTDVDVGPFLNSLVQEGEWERAAAVALFNLDIRRAIQILNEGASSEKGDLNLNVVAMALSGYTDEKNSLWREMCSTLRLQLNNPYLCVMFAFLTSEAGAYDGVLYENKVAVRDRVAFACKFLGDAQLNKYIEKLTNEMKEAGNLEGILLTGLTKDGVDLMESYVDRTGDVQTASYCMLQGSPLDVLKDERVQYWIENYRNLLDAWRFWHKRAEFDIHRSKLDPSSKPLAQVFVSCNFCGKSISYSCSSVPHQGRGFSQYGVSGSPTKSKVTSCPGCRKPLPRCALCLINMGTPVSSCPGGSKSDEKVDLSKDKKLAQFNNWFTWCHNCRHGGHAGHMLSWFRDHAECPVSACTCKCMQLDTTGNLVPAETVQP
uniprot
proteins
function
FUNCTION: As a component of the GATOR2 complex, functions as an activator of the amino acid-sensing branch of the mTORC1 signaling pathway (PubMed:35022234). The GATOR2 complex indirectly activates mTORC1 through the inhibition of the GATOR1 subcomplex (By similarity). GATOR2 probably acts as an E3 ubiquitin-protein ligase toward GATOR1 (By similarity). In the presence of abundant amino acids, the GATOR2 complex mediates ubiquitination of the NPRL2 core component of the GATOR1 complex, leading to GATOR1 inactivation (By similarity). In the absence of amino acids, GATOR2 is inhibited, activating the GATOR1 complex (By similarity). Within the GATOR2 complex, MIOS is required to prevent autoubiquitination of WDR24, the catalytic subunit of the complex (By similarity). The GATOR2 complex is required for brain myelination (PubMed:35022234). {ECO:0000250|UniProtKB:Q9NXC5, ECO:0000269|PubMed:35022234}.
uniprot
proteins
mass_dalton
98335
uniprot
proteins
protein_family
WD repeat mio family
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
19468303
uniprot
proteins
pubmed
21183079
uniprot
proteins
pubmed
35022234
uniprot
proteins
sequence_length
875
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Lysosome membrane {ECO:0000250|UniProtKB:Q9NXC5}.
uniprot
proteins