Rabbit Anti-C2a/FITC Conjugated antibody

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Complement C2a fragment; C2; C2 protein; C3/C5 convertase; CO 2; CO2; complement C2; Complement component 2; complement component C2; DKFZp779M0311; OTTHUMP00000062690; CO2_HUMAN.

Cat:

SL10428R-FITC

Species Reactivity：

(predicted: Human,)

Immunogen：

KLH conjugated synthetic peptide derived from human C2a

Format：

Lyophilized or Liquid

Storage instructions：

Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of ant

Buffer：

0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Calculated MW：

56kDa

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Unit:

Price: $596.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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background:
Component C2 is a serum glycoprotein that functions as part of the classical pathway of the complement system. Activated C1 cleaves C2 into C2a and C2b. The serine proteinase C2a then combines with complement factor 4b to create the C3 or C5 convertase. Deficiency of C2 has been reported to associated with certain autoimmune diseases and SNPs in this gene have been associated with altered susceptibility to age-related macular degeneration. This gene localizes within the class III region of the MHC on the short arm of chromosome 6. Alternative splicing results in multiple transcript variants encoding distinct isoforms. Additional transcript variants have been described in publications but their full-length sequence has not been determined.[provided by RefSeq, Mar 2009].

Function:
Component C2 which is part of the classical pathway ofthe complement system is cleaved by activated factor C1 into twofragments: C2b and C2a. C2a, a serine protease, then combines withcomplement factor 4b to generate the C3 or C5 convertase.

Subunit:
C2a interacts with Schistosoma haematobium TOR (viaN-terminal extracellular domain). This results in inhibition of theclassical and lectin pathway of complement activation, probably dueto interference with binding of C2a to C4b such that C3 convertasecannot be formed. This infers resistance to complement-mediatedcell lysis, allowing parasite survival and infection.

Subcellular Location:
Secreted.

DISEASE:
Defects in C2 are the cause of complement component 2deficiency (C2D) [MIM:217000]. A deficiency of the complementclassical pathway associated with the development of autoimmunedisorders, mainly systemic lupus erythematosus. Skin and jointmanifestations are common and renal disease is relatively rare.Patients with complement component 2 deficiency are also reportedto have recurrent or invasive infections.

Similarity:
Belongs to the peptidase S1 family.
Contains 1 peptidase S1 domain.
Contains 3 Sushi (CCP/SCR) domains.
Contains 1 VWFA domain.

Database links:

Entrez Gene: 717 Human

Omim: 217000 Human

SwissProt: P06681 Human

Unigene: 408903 Human

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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