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MASP1

This gene encodes a serine protease that functions as a component of the lectin pathway of complement activation. The complement pathway plays an essential role in the innate and adaptive immune response. The encoded protein is synthesized as a zymogen and is activated when it complexes with the pathogen recognition molecules of lectin pathway, the mannose-binding lectin and the ficolins. This protein is not directly involved in complement activation but may play a role as an amplifier of complement activation by cleaving complement C2 or by activating another complement serine protease, MASP-2. The encoded protein is also able to cleave fibrinogen and factor XIII and may may be involved in coagulation. A splice variant of this gene which lacks the serine protease domain functions as an inhibitor of the complement pathway. Alternate splicing results in multiple transcript variants.[provided by RefSeq, Apr 2010]
Full Name
Mannan Binding Lectin Serine Peptidase 1
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. Also plays a role in development (PubMed:21258343).
Biological Process
Cell surface pattern recognition receptor signaling pathway Source: ComplexPortal
Complement activation, lectin pathway Source: ComplexPortal
Negative regulation of complement activation Source: UniProtKB
Positive regulation of opsonization Source: ComplexPortal
Proteolysis Source: ComplexPortal
Cellular Location
Secreted
Involvement in disease
3MC syndrome 1 (3MC1):
A form of 3MC syndrome, an autosomal recessive disorder characterized by facial dysmorphism, craniosynostosis, learning disability, and genital, limb and vesicorenal anomalies. Facial features include hypertelorism, blepharophimosis, blepharoptosis and highly arched eyebrows, cleft lip and/or palate. The term 3MC syndrome includes Carnevale, Mingarelli, Malpuech, and Michels syndromes.
PTM
The iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains.
N-glycosylated. Some N-linked glycan are of the complex-type (By similarity).
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.

Anti-MASP1 antibodies

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Target: MASP1
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human
Clone: 2D5B6
Application*: IA, IP, P, WB
Target: MASP1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBFYM-1737
Application*: IP, WB
Target: MASP1
Host: Rat
Antibody Isotype: IgG2a
Specificity: Human
Clone: CBFYM-1738
Application*: IA, WB
Target: MASP1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Rat
Clone: CBFYM-1741
Application*: P, WB
Target: MASP1
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human
Clone: CBFYA-0270
Application*: IA, IP, P, WB
Target: MASP1
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human
Clone: CBHb313
Application*: Immuno assays, IP, IHC-P, WB
Target: MASP1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBHb314
Application*: Immuno assays
Target: MASP1
Host: Rat
Antibody Isotype: IgG2a
Specificity: Human
Clone: CBFYM-1739
Application*: E, WB
Target: MASP1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBFYM-1740
Application*: IA
Target: MASP1
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human
Clone: CBFYC-2938
Application*: WB
Target: MASP1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBFYA-0111
Application*: IA, IP, WB
Target: MASP1
Host: Rat
Antibody Isotype: IgG2a
Specificity: Human
Clone: CBFYA-0174
Application*: IA, WB
Target: MASP1
Host: Mouse
Antibody Isotype: IgG2
Specificity: Human
Clone: CBYY-C3042
Application*: WB
Target: MASP1
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBXC-2929
Application*: WB
More Infomation
For Research Use Only. Not For Clinical Use.
(P): Predicted
* Abbreviations
IFImmunofluorescence
IHImmunohistochemistry
IPImmunoprecipitation
WBWestern Blot
EELISA
MMicroarray
CIChromatin Immunoprecipitation
FFlow Cytometry
FNFunction Assay
IDImmunodiffusion
RRadioimmunoassay
TCTissue Culture
GSGel Supershift
NNeutralization
BBlocking
AActivation
IInhibition
DDepletion
ESELISpot
DBDot Blot
MCMass Cytometry/CyTOF
CTCytotoxicity
SStimulation
AGAgonist
APApoptosis
IMImmunomicroscopy
BABioassay
CSCostimulation
EMElectron Microscopy
IEImmunoelectrophoresis
PAPeptide Array
ICImmunocytochemistry
PEPeptide ELISA
MDMeDIP
SHIn situ hybridization
IAEnzyme Immunoassay
SEsandwich ELISA
PLProximity Ligation Assay
ECELISA(Cap)
EDELISA(Det)
BIBioimaging
IOImmunoassay
LFLateral Flow Immunoassay
LALuminex Assay
CImmunohistochemistry-Frozen Sections
PImmunohistologyp-Paraffin Sections
ISIntracellular Staining for Flow Cytometry
MSElectrophoretic Mobility Shift Assay
RIRNA Binding Protein Immunoprecipitation (RIP)
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