IRGQ
entrez
126298
canonical
uniprot
Q8WZA9
canonical
ensembl
ENSG00000167378
resolver
ensp
ENSP00000387535
resolver
ensp
ENSP00000472250
resolver
ensp
ENSP00000609592
resolver
ensp
ENSP00000642124
resolver
enst
ENST00000422989
resolver
enst
ENST00000602269
resolver
enst
ENST00000939533
resolver
enst
ENST00000972065
resolver
genesymbol
IRGQ
resolver
genesymbol_syn
IRGQ1
resolver
hgnc
HGNC:24868
resolver
uniprot
B2RNP3
resolver
uniprot_entry
IRGQ_HUMAN
resolver
uniprot_sec
B2RNP3
resolver
amino_acid_sequence
MPPPQGDVTALFLGPPGLGKSALIAALCDKDVETLEAPEGRPDSGVPSLRAAGPGLFLGELSCPPAAPGPWAAEANVLVLVLPGPEGNGEPLAPALGEAALAALARGTPLLAVRNLRPGDSQTAAQARDQTAALLNSAGLGAADLFVLPANCGSSDGCEELERLRAALQSQAEALRRLLPPAQDGFEVLGAAELEAVREAFETGGLEAALSWVRSGLERLGSARLDLAVAGKADVGLVVDMLLGLDPGDPGAAPASVPTAPTPFPAPERPNVVLWTVPLGHTGTATTAAAASHPTHYDALILVTPGAPTEKDWAQVQALLLPDAPLVCVRTDGEGEDPECLGEGKMENPKGESLKNAGGGGLENALSKGREKCSAGSQKAGSGEGPGKAGSEGLQQVVGMKKSGGGDSERAAALSPEDETWEVLEEAPPPVFPLRPGGLPGLCEWLRRALPPAQAGALLLALPPASPSAARTKAAALRAGAWRPALLASLAAAAAPLPGLGWACDVALLRGQLAEWRRGLGLEPTALARRERALGLASGELAARAHFPGPVTRAEVEARLGAWAGEGTAGGAALGALSFLWPAGGAAATGGLGYRAAHGVLLQALDEMRADAEAVLAPPEPAQ
uniprot
proteins
function
FUNCTION: Autophagy receptor that specifically promotes clearance of misfolded MHC class I molecules by targeting them to the lysosome for degradation (PubMed:39481378). Acts as a molecular adapter that specifically recognizes and binds (1) misfolded MHC class I molecules following their ubiquitination, as well as (2) autophagy-related proteins, promoting the recruitment of misfolded MHC class I molecules to autophagy machinery for degradation (PubMed:39481378). Degradation of misfolded MHC class I molecules is essential to prevent accumulation of defective MHC class I complexes at the surface of CD8(+) T-cells and prevent a stronger T-cell-mediated response (PubMed:39481378). In contrast to other members of the family, does not show GTPase activity (PubMed:39481378). {ECO:0000269|PubMed:39481378}.
uniprot
proteins
mass_dalton
62717
uniprot
proteins
mutagenesis
MUTAGEN 74; /note="E->R: Abolished interaction with GABARAPL2."; /evidence="ECO:0000269|PubMed:39481378
uniprot
proteins
protein_family
IRG family
uniprot
proteins
protein_family
TRAFAC class dynamin-like GTPase superfamily
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
18669648
uniprot
proteins
pubmed
21269460
uniprot
proteins
pubmed
24275569
uniprot
proteins
pubmed
39481378
uniprot
proteins
sequence_length
623
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Lysosome {ECO:0000269|PubMed:39481378}. Cytoplasmic vesicle, autophagosome {ECO:0000269|PubMed:39481378}.
uniprot
proteins