O35658
uniprot
O35658
canonical
amino_acid_sequence
MLPLLRCVPRSLGAASGLRTAIPAQPLRHLLQPAPRPCLRPFGLLSVRAGSARRSGLLQPPVPCACGCGALHTEGDKAFVEFLTDEIKEEKKIQKHKSLPKMSGDWELEVNGTEAKLLRKVAGEKITVTFNINNSIPPTFDGEEEPSQGQKAEEQEPERTSTPNFVVEVTKTDGKKTLVLDCHYPEDEIGHEDEAESDIFSIKEVSFQATGDSEWRDTNYTLNTDSLDWALYDHLMDFLADRGVDNTFADELVELSTALEHQEYITFLEDLKSFVKNQ
uniprot
proteins
function
FUNCTION: Is believed to be a multifunctional and multicompartmental protein involved in inflammation and infection processes, ribosome biogenesis, protein synthesis in mitochondria, regulation of apoptosis, transcriptional regulation and pre-mRNA splicing. At the cell surface is thought to act as an endothelial receptor for plasma proteins of the complement and kallikrein-kinin cascades. Putative receptor for C1q; specifically binds to the globular 'heads' of C1q thus inhibiting C1; may perform the receptor function through a complex with C1qR/CD93 (PubMed:9414106). In complex with cytokeratin-1/KRT1 is a high affinity receptor for kininogen-1/HMWK. Can also bind other plasma proteins, such as coagulation factor XII leading to its autoactivation. May function to bind initially fluid kininogen-1 to the cell membrane. The secreted form may enhance both extrinsic and intrinsic coagulation pathways. It is postulated that the cell surface form requires docking with transmembrane proteins for downstream signaling which might be specific for a cell-type or response. By acting as C1q receptor is involved in chemotaxis of immature dendritic cells and neutrophils and is proposed to signal through CD209/DC-SIGN on immature dendritic cells, through integrin alpha-4/beta-1 during trophoblast invasion of the decidua, and through integrin beta-1 during endothelial cell adhesion and spreading. Signaling involved in inhibition of innate immune response is implicating the PI3K-AKT/PKB pathway. Required for protein synthesis in mitochondria (PubMed:22904065, PubMed:28942965). In mitochondrial translation may be involved in formation of functional 55S mitoribosomes; the function seems to involve its RNA-binding activity (PubMed:22904065, PubMed:28942965). Acts as a RNA modification reader, which specifically recognizes and binds mitochondrial RNAs modified by C5-methylcytosine (m5C) in response to stress, and promotes recruitment of the mitochondrial degradosome complex, leading to their degradation (By similarity). May be involved in the nucleolar ribosome maturation process; the function may involve the exchange of FBL for RRP1 in the association with pre-ribosome particles. Involved in regulation of RNA splicing by inhibiting the RNA-binding capacity of SRSF1 and its phosphorylation. Is required for the nuclear translocation of splicing factor U2AF1L4 (PubMed:18460468). Involved in regulation of CDKN2A- and HRK-mediated apoptosis. May be involved in regulation of FOXC1 transcriptional activity and NFY/CCAAT-binding factor complex-mediated transcription. May play a role in antibacterial defense (By similarity). Acts as a regulator of DNA repair via homologous recombination by inhibiting the activity of MRE11: interacts with unphosphorylated MRE11 and RAD50 in absence of DNA damage, preventing formation and activity of the MRN complex (By similarity). Following DNA damage, dissociates from phosphorylated MRE11, allowing formation of the MRN complex (By similarity). {ECO:0000250|UniProtKB:Q07021, ECO:0000269|PubMed:17486078, ECO:0000269|PubMed:18166172, ECO:0000269|PubMed:18460468, ECO:0000269|PubMed:22904065, ECO:0000269|PubMed:28942965}.
uniprot
proteins
mass_dalton
31013
uniprot
proteins
mutagenesis
MUTAGEN 88; /note="K->A: Impairs RNA binding and mitochondrial translation; when associated with A-92."; /evidence="ECO:0000269|PubMed:22904065"; MUTAGEN 92; /note="K->A: Impairs RNA binding and mitochondrial translation; when associated with A-88."; /evidence="ECO:0000269|PubMed:22904065"; MUTAGEN 243; /note="G->W: Partially able to rescue mitochondrial respiratory chain defects observed in knockout mice."; /evidence="ECO:0000269|PubMed:28942965"; MUTAGEN 271; /note="L->P: Not able to rescue mitochondrial respiratory chain defects observed in knockout mice."; /evidence="ECO:0000269|PubMed:28942965
uniprot
proteins
protein_family
MAM33 family
uniprot
proteins
pubmed
17486078
uniprot
proteins
pubmed
18166172
uniprot
proteins
pubmed
18460468
uniprot
proteins
pubmed
20950273
uniprot
proteins
pubmed
21183079
uniprot
proteins
pubmed
22904065
uniprot
proteins
pubmed
23576753
uniprot
proteins
pubmed
28942965
uniprot
proteins
pubmed
9414106
uniprot
proteins
sequence_length
278
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Mitochondrion matrix {ECO:0000269|PubMed:17486078, ECO:0000269|PubMed:18166172}. Nucleus {ECO:0000250|UniProtKB:Q07021}. Cell membrane {ECO:0000250|UniProtKB:Q07021}; Peripheral membrane protein {ECO:0000250|UniProtKB:Q07021}; Extracellular side {ECO:0000250|UniProtKB:Q07021}. Secreted {ECO:0000250|UniProtKB:Q07021}. Cytoplasm {ECO:0000250|UniProtKB:Q07021}. Nucleus, nucleolus {ECO:0000250|UniProtKB:Q07021}. Note=Seems to be predominantly localized to mitochondria (PubMed:17486078, PubMed:18166172). Secreted by activated lymphocytes (By similarity). {ECO:0000250|UniProtKB:Q07021, ECO:0000269|PubMed:17486078, ECO:0000269|PubMed:18166172}.
uniprot
proteins