KCMF1
entrez
56888
canonical
uniprot
Q9P0J7
canonical
ensembl
ENSG00000176407
resolver
ensp
ENSP00000386738
resolver
ensp
ENSP00000537204
resolver
ensp
ENSP00000537205
resolver
ensp
ENSP00000626622
resolver
ensp
ENSP00000626623
resolver
ensp
ENSP00000626624
resolver
ensp
ENSP00000626625
resolver
enst
ENST00000409785
resolver
enst
ENST00000867145
resolver
enst
ENST00000867146
resolver
enst
ENST00000956563
resolver
enst
ENST00000956564
resolver
enst
ENST00000956565
resolver
enst
ENST00000956566
resolver
genesymbol
KCMF1
resolver
genesymbol_syn
FIGC
resolver
genesymbol_syn
ZZZ1
resolver
hgnc
HGNC:20589
resolver
uniprot
Q4ZG04
resolver
uniprot
Q53SC7
resolver
uniprot
Q9BWK2
resolver
uniprot
Q9H8P5
resolver
uniprot
Q9UFE8
resolver
uniprot_entry
KCMF1_HUMAN
resolver
uniprot_sec
Q4ZG04
resolver
uniprot_sec
Q53SC7
resolver
uniprot_sec
Q9BWK2
resolver
uniprot_sec
Q9H8P5
resolver
uniprot_sec
Q9UFE8
resolver
amino_acid_sequence
MSRHEGVSCDACLKGNFRGRRYKCLICYDYDLCASCYESGATTTRHTTDHPMQCILTRVDFDLYYGGEAFSVEQPQSFTCPYCGKMGYTETSLQEHVTSEHAETSTEVICPICAALPGGDPNHVTDDFAAHLTLEHRAPRDLDESSGVRHVRRMFHPGRGLGGPRARRSNMHFTSSSTGGLSSSQSSYSPSNREAMDPIAELLSQLSGVRRSAGGQLNSSGPSASQLQQLQMQLQLERQHAQAARQQLETARNATRRTNTSSVTTTITQSTATTNIANTESSQQTLQNSQFLLTRLNDPKMSETERQSMESERADRSLFVQELLLSTLVREESSSSDEDDRGEMADFGAMGCVDIMPLDVALENLNLKESNKGNEPPPPPL
uniprot
proteins
ec_number
2.3.2.27
uniprot
proteins
function
FUNCTION: E3 ubiquitin-protein ligase which accepts ubiquitin from an E2 ubiquitin-conjugating enzyme and then transfers it to targeted substrates, promoting their degradation by the proteasome (PubMed:15581609, PubMed:25582440, PubMed:34893540, PubMed:37891180, PubMed:38297121). Together with UBR4, component of the N-end rule pathway: ubiquitinates proteins bearing specific N-terminal residues that are destabilizing according to the N-end rule, leading to their degradation (PubMed:34893540, PubMed:37891180). Does not ubiquitinate proteins that are acetylated at the N-terminus (PubMed:37891180). Together with UBR4, part of a protein quality control pathway that catalyzes ubiquitination and degradation of proteins that have been oxidized in response to reactive oxygen species (ROS): recognizes proteins with an Arg-CysO3(H) degron at the N-terminus, and mediates assembly of heterotypic 'Lys-63'-/'Lys-27'-linked branched ubiquitin chains on oxidized proteins, leading to their degradation by autophagy (PubMed:34893540). Catalytic component of the silencing factor of the integrated stress response (SIFI) complex, a multiprotein complex that turns off the mitochondrial stress response after a specific stress event has been resolved (PubMed:38297121, PubMed:40328314, PubMed:40875847). The SIFI complex ubiquitinates and degrades (1) components of the HRI-mediated signaling of the integrated stress response, such as DELE1 and EIF2AK1/HRI, as well as (2) unimported mitochondrial precursors such as ABHD10 (PubMed:38297121, PubMed:40328314). Within the SIFI complex, UBR4 initiates ubiquitin chain that are further elongated or branched by KCMF1 (PubMed:38297121, PubMed:40328314, PubMed:40875847). {ECO:0000269|PubMed:15581609, ECO:0000269|PubMed:25582440, ECO:0000269|PubMed:34893540, ECO:0000269|PubMed:37891180, ECO:0000269|PubMed:38297121, ECO:0000269|PubMed:40328314, ECO:0000269|PubMed:40875847}.
uniprot
proteins
mass_dalton
41945
uniprot
proteins
mutagenesis
MUTAGEN 29; /note="D->R: Abolishes interaction with ABHD10; when associated with R-31."; /evidence="ECO:0000269|PubMed:40328314"; MUTAGEN 31; /note="D->R: Abolishes interaction with ABHD10; when associated with R-29."; /evidence="ECO:0000269|PubMed:40328314"; MUTAGEN 61; /note="Missing: Abolishes interaction with ABHD10."; /evidence="ECO:0000269|PubMed:40328314"; MUTAGEN 318; /note="L->A: Disrupts UBR4 dimerization within the SIFI complex; when associated with A-319 and S-320."; /evidence="ECO:0000269|PubMed:40875847"; MUTAGEN 319; /note="F->A: Disrupts UBR4 dimerization within the SIFI complex; when associated with A-318 and S-320."; /evidence="ECO:0000269|PubMed:40875847"; MUTAGEN 320; /note="V->S: Disrupts UBR4 dimerization within the SIFI complex; when associated with A-318 and A-319."; /evidence="ECO:0000269|PubMed:40875847
uniprot
proteins
pathway_participation
PATHWAY: Protein modification; protein ubiquitination. {ECO:0000269|PubMed:25582440, ECO:0000269|PubMed:34893540, ECO:0000269|PubMed:37891180, ECO:0000269|PubMed:38297121, ECO:0000269|PubMed:40328314, ECO:0000269|PubMed:40875847}.
uniprot
proteins
protein_family
KCMF1 family
uniprot
proteins
pubmed
14702039
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
15581609
uniprot
proteins
pubmed
17974005
uniprot
proteins
pubmed
18669648
uniprot
proteins
pubmed
18691976
uniprot
proteins
pubmed
20068231
uniprot
proteins
pubmed
21269460
uniprot
proteins
pubmed
23186163
uniprot
proteins
pubmed
24275569
uniprot
proteins
pubmed
25582440
uniprot
proteins
pubmed
34893540
uniprot
proteins
pubmed
37891180
uniprot
proteins
pubmed
38297121
uniprot
proteins
pubmed
40328314
uniprot
proteins
pubmed
40875847
uniprot
proteins
sequence_length
381
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Cytoplasm {ECO:0000269|PubMed:38297121}. Late endosome {ECO:0000269|PubMed:25582440}. Lysosome {ECO:0000269|PubMed:25582440}.
uniprot
proteins