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C1S

This gene encodes a serine protease, which is a major constituent of the human complement subcomponent C1. C1s associates with two other complement components C1r and C1q in order to yield the first component of the serum complement system. Defects in this gene are the cause of selective C1s deficiency.
Full Name
complement C1s
Function
C1s B chain is a serine protease that combines with C1q and C1r to form C1, the first component of the classical pathway of the complement system. C1r activates C1s so that it can, in turn, activate C2 and C4.
Biological Process
Complement activation Source: Reactome
Complement activation, classical pathway Source: Reactome
Innate immune response Source: UniProtKB-KW
Regulation of complement activation Source: Reactome
Cellular Location
Blood microparticle; Extracellular region; Extracellular space
Involvement in disease
Complement component C1s deficiency (C1SD): A rare defect resulting in C1 deficiency and impaired activation of the complement classical pathway. C1 deficiency generally leads to severe immune complex disease with features of systemic lupus erythematosus and glomerulonephritis.
Ehlers-Danlos syndrome, periodontal type, 2 (EDSPD2):A form of Ehlers-Danlos syndrome, a connective tissue disorder characterized by hyperextensible skin, atrophic cutaneous scars due to tissue fragility and joint hyperlaxity. EDSPD2 is characterized by the association of typical features of Ehlers-Danlos syndrome with gingival recession and severe early-onset periodontal disease, leading to premature loss of permanent teeth. EDSPD2 transmission pattern is consistent with autosomal dominant inheritance.
PTM
The iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains.
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Anti-C1S antibodies

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Mouse Anti-C1S Recombinant Antibody (CBLC019-LY) (CBMAB-C9527-LY)

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Target: C1S
Host: Mouse
Antibody Isotype: IgG2b, ĸ
Specificity: Human
Clone: CBLC019-LY
Application*: WB, IP, IF, E
Datasheet SDS

Mouse Anti-C1S Recombinant Antibody (04) (CBMAB-C1737-LY)

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Target: C1S
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: 04
Application*: E
Datasheet SDS

Mouse Anti-C1S Recombinant Antibody (9) (CBMAB-C9295-LY)

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Target: C1S
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: 9
Application*: E, IH
Datasheet SDS

Mouse Anti-C1S Recombinant Antibody (306904) (CBMAB-C5462-LY)

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Target: C1S
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: 306904
Application*: IP, WB
Datasheet SDS

Rabbit Anti-C1S Recombinant Antibody (006) (CBMAB-C1529-LY)

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Target: C1S
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: 006
Application*: E
Datasheet SDS

Mouse Anti-C1S Recombinant Antibody (M81) (CBMAB-C1126-CN)

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Target: C1S
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: M81
Application*: WB, IP, E, IH, FC
Datasheet SDS

Mouse Anti-C1S Recombinant Antibody (M241) (CBMAB-C1125-CN)

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Target: C1S
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human
Clone: M241
Application*: WB, IP, E, IH, FC
Datasheet SDS

Rabbit Anti-C1S Recombinant Antibody (CBFYC-0612) (CBMAB-C0666-FY)

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Target: C1S
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: CBFYC-0612
Application*: WB, IP, IH
Datasheet SDS
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(P): Predicted
* Abbreviations
  • AActivation
  • AGAgonist
  • APApoptosis
  • BBlocking
  • BABioassay
  • BIBioimaging
  • CImmunohistochemistry-Frozen Sections
  • CIChromatin Immunoprecipitation
  • CTCytotoxicity
  • CSCostimulation
  • DDepletion
  • DBDot Blot
  • EELISA
  • ECELISA(Cap)
  • EDELISA(Det)
  • ESELISpot
  • EMElectron Microscopy
  • FFlow Cytometry
  • FNFunction Assay
  • GSGel Supershift
  • IInhibition
  • IAEnzyme Immunoassay
  • ICImmunocytochemistry
  • IDImmunodiffusion
  • IEImmunoelectrophoresis
  • IFImmunofluorescence
  • IHImmunohistochemistry
  • IMImmunomicroscopy
  • IOImmunoassay
  • IPImmunoprecipitation
  • ISIntracellular Staining for Flow Cytometry
  • LALuminex Assay
  • LFLateral Flow Immunoassay
  • MMicroarray
  • MCMass Cytometry/CyTOF
  • MDMeDIP
  • MSElectrophoretic Mobility Shift Assay
  • NNeutralization
  • PImmunohistologyp-Paraffin Sections
  • PAPeptide Array
  • PEPeptide ELISA
  • PLProximity Ligation Assay
  • RRadioimmunoassay
  • SStimulation
  • SESandwich ELISA
  • SHIn situ hybridization
  • TCTissue Culture
  • WBWestern Blot
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