Factor I rabbit pAb

Name: Factor I rabbit pAb
Brand: ELK Biotechnology
Price: 148.0 USD
Availability: InStock

Vendor: ELK Biotechnology

SKU: ES5825

$148.00 USD

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For research use only — not intended for clinical or diagnostic purposes.

Product Overview

Applications

WB;ELISA

Cellular localization

Secreted, extracellular space. Secreted .

Clonality

Polyclonal

Concentration

1 mg/ml

Geneid human

3426

Human swiss prot no

P05156

Immunogen

The antiserum was produced against synthesized peptide derived from human CFI. AA range:441-490

Isotype

IgG

Observed band

Full lenth:66kD, heavy chain: 50-58kD

Product weight

0.005kg

Reactivity

Human;Rat;Mouse;

Recommended dilutions

IHC: 1/500 - 1/2000. ELISA: 1/10000. Not yet tested in other applications.

Source

Rabbit

Storage

-20°C/1 year

Background

This gene encodes a serine proteinase that is essential for regulating the complement cascade. The encoded preproprotein is cleaved to produce both heavy and light chains, which are linked by disulfide bonds to form a heterodimeric glycoprotein. This heterodimer can cleave and inactivate the complement components C4b and C3b, and it prevents the assembly of the C3 and C5 convertase enzymes. Defects in this gene cause complement factor I deficiency, an autosomal recessive disease associated with a susceptibility to pyogenic infections. Mutations in this gene have been associated with a predisposition to atypical hemolytic uremic syndrome, a disease characterized by acute renal failure, microangiopathic hemolytic anemia and thrombocytopenia. Primary glomerulonephritis with immune deposits and age-related macular degeneration are other conditions associated with mutations of this gene. [provided by Ref

Factor I rabbit pAb

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