Rabbit Anti-Factor I heavy chain/FITC Conjugated antibody

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Complement factor I heavy chain; Factor I heavy chain; AHUS3; C3b INA; C3b inactivator; C3B/C4B inactivator; C3BINA; CFAI_HUMAN; CFI; Complement component I; Complement control protein factor I; Complement factor I; F1; factor I; FactorI; FI; I factor; IF

Cat:

SL10339R-FITC

Species Reactivity：

(predicted: Human,Mouse,Rat,)

Immunogen：

KLH conjugated synthetic peptide derived from human Complement factor I heavy chain

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：

ICC=1:50-200IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Calculated MW：

35/63kDa

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Unit:

Price: $596.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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background:
The complement pathway is an important host defense system that contributes to both innate and acquired immunity. There are three pathways of complement activation: the classical pathway, lectin pathway and alternative pathway. Complement protein Factor I is a key serine protease that modulates the complement cascade by regulating the levels of C3 convertases. It circulates in plasma as a heavily N-glycosylated heterodimer made up of two disulfide linked chains, each carrying three N-linked oligosaccharide chains that may have both structural and functional roles in the interactions with the natural substrate and the cofactor during catalysis. Factor I is a serine protease with a high degree of specificity for C3b and C4b. It requires protein cofactors for cleavage of these complement proteins; Factor H, CR1 or MCP are required for C3b cleavage, and C4bp or CR1 are required for C4b cleavage.

Function:
Responsible for cleaving the alpha-chains of C4b and C3b in the presence of the cofactors C4-binding protein and factor H respectively.

Subcellular Location:
Secreted; extracellular space.

Tissue Specificity:
Plasma.

DISEASE:
Defects in CFI are a cause of susceptibility to hemolytic uremic syndrome atypical type 3 (AHUS3) [MIM:612923]. An atypical form of hemolytic uremic syndrome. It is a complex genetic disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, renal failure and absence of episodes of enterocolitis and diarrhea. In contrast to typical hemolytic uremic syndrome, atypical forms have a poorer prognosis, with higher death rates and frequent progression to end-stage renal disease. Note=Susceptibility to the development of atypical hemolytic uremic syndrome can be conferred by mutations in various components of or regulatory factors in the complement cascade system. Other genes may play a role in modifying the phenotype. Defects in CFI are the cause of complement factor I deficiency (CFI deficiency) [MIM:62204]. CFI deficiency is an autosomal recessive condition associated with a propensity to pyogenic infections.

Similarity:
Belongs to the peptidase S1 family.
Contains 1 Kazal-like domain.
Contains 2 LDL-receptor class A domains.
Contains 1 peptidase S1 domain.

Database links:

Entrez Gene: 3426 Human

Omim: 217030 Human

SwissProt: P05156 Human

Unigene: 312485 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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