Statements in which the resource exists as a subject.
PredicateObject
rdf:type
biopax3:comment
FUNCTION: Functions in the lectin pathway of complement, which performs a key role in innate immunity by recognizing pathogens through patterns of sugar moieties and neutralizing them. The lectin pathway is triggered upon binding of mannan-binding lectin (MBL) and ficolins to sugar moieties which leads to activation of the associated proteases MASP1 and MASP2. Functions as an endopeptidase and may activate MASP2 or C2 or directly activate C3 the key component of complement reaction. Isoform 2 may have an inhibitory effect on the activation of the lectin pathway of complement or may cleave IGFBP5. ENZYME REGULATION: Inhibited by SERPING1 and A2M (By similarity). SUBUNIT: Homodimer. Interacts with the oligomeric lectins MBL1, MBL2, FCN2 and FCN3; triggers the lectin pathway of complement through activation of C3. Interacts with SERPING1 (By similarity). SUBCELLULAR LOCATION: Secreted. ALTERNATIVE PRODUCTS: Event=Alternative splicing; Named isoforms=2; Name=1; Synonyms=MASP-1; IsoId=P98064-1; Sequence=Displayed; Name=2; Synonyms=MASP-3; IsoId=P98064-2; Sequence=VSP_036814, VSP_036815; Note=Glycosylated on Asn-538 and Asn-604 (By similarity); TISSUE SPECIFICITY: Protein of the plasma which is primarily expressed by liver. PTM: The iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains (By similarity). PTM: N-glycosylated. Some N-linked glycan are of the complex-type (By similarity). PTM: Autoproteolytic processing of the proenzyme produces the active enzyme composed on the heavy and the light chain held together by a disulfide bond. Isoform 1 but not isoform 2 is activated through autoproteolytic processing (By similarity). DISRUPTION PHENOTYPE: Mice are smaller and more vulnerable indicating developmental and growth defects. Mice serum has low C4 and C3 cleavage activity together with low MASP2 activation. SIMILARITY: Belongs to the peptidase S1 family. SIMILARITY: Contains 2 CUB domains. SIMILARITY: Contains 1 EGF-like domain. SIMILARITY: Contains 1 peptidase S1 domain. SIMILARITY: Contains 2 Sushi (CCP/SCR) domains. GENE SYNONYMS:Masp1 Crarf Masp3. COPYRIGHT: Protein annotation is derived from the UniProt Consortium (http://www.uniprot.org/). Distributed under the Creative Commons Attribution-NoDerivs License., SEQUENCE 704 AA; 79968 MW; 4CF0B17916C10961 CRC64;
biopax3:xref
biopax3:displayName
MASP1_MOUSE
biopax3:name
3.4.21.-, Complement factor MASP-3, Complement-activating component of Ra-reactive factor, MASP-1, Mannan-binding lectin serine protease 1 heavy chain, Mannan-binding lectin serine protease 1 light chain, Mannose-binding lectin-associated serine protease 1, Mannose-binding protein-associated serine protease, Masp1, Ra-reactive factor serine protease p100, RaRF, Serine protease 5
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biopax3:organism
biopax3:sequence
MRFLSFWRLLLYHALCLALPEVSAHTVELNEMFGQIQSPGYPDSYPSDSEVTWNITVPEGFRIKLYFMHFNLESSYLCEYDYVKVETEDQVLATFCGRETTDTEQTPGQEVVLSPGTFMSVTFRSDFSNEERFTGFDAHYMAVDVDECKEREDEELSCDHYCHNYIGGYYCSCRFGYILHTDNRTCRVECSGNLFTQRTGTITSPDYPNPYPKSSECSYTIDLEEGFMVSLQFEDIFDIEDHPEVPCPYDYIKIKAGSKVWGPFCGEKSPEPISTQTHSVQILFRSDNSGENRGWRLSYRAAGNECPKLQPPVYGKIEPSQAVYSFKDQVLVSCDTGYKVLKDNEVMDTFQIECLKDGAWSNKIPTCKIVDCGAPAGLKHGLVTFSTRNNLTTYKSEIRYSCQQPYYKMLHNTTGVYTCSAHGTWTNEVLKRSLPTCLPVCGVPKFSRKQISRIFNGRPAQKGTMPWIAMLSHLNGQPFCGGSLLGSNWVLTAAHCLHQSLDPEEPTLHSSYLLSPSDFKIIMGKHWRRRSDEDEQHLHVKRTTLHPLYNPSTFENDLGLVELSESPRLNDFVMPVCLPEQPSTEGTMVIVSGWGKQFLQRFPENLMEIEIPIVNSDTCQEAYTPLKKKVTKDMICAGEKEGGKDACAGDSGGPMVTKDAERDQWYLVGVVSWGEDCGKKDRYGVYSYIYPNKDWIQRITGVRN
biopax3:standardName
Mannan-binding lectin serine protease 1