CFH, Polyclonal Antibody

Complement factor H; H factor 1; CFH; HF; HF1; HF2

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit Ig

Rabbit

This CFH antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 751-780 amino acids from the Central region of human CFH.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.287 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-CFH antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

This gene is a member of the Regulator of Complement
Activation (RCA) gene cluster and encodes a protein with twenty
short concensus repeat (SCR) domains. This protein is secreted into
the bloodstream and has an essential role in the regulation of
complement activation, restricting this innate defense mechanism to
microbial infections. Mutations in this gene have been associated
with hemolytic-uremic syndrome (HUS) and chronic hypocomplementemic
nephropathy. Alternate transcriptional splice variants, encoding
different isoforms, have been characterized.

Immunology

Western Blot (WB), ELISA (EIA), Immunohistochemistry (IHC), Immunofluorescence (IF)

WB~~1:1000

CFH Antibody (Center) western blot analysis in K562 cell line lysates (35ug/lane).This demonstrates the CFH antibody detected the CFH protein (arrow).

CFH antibody (Center) immunohistochemistry analysis in formalin fixed and paraffin embedded human hepatocarcinoma followed by peroxidase conjugation of the secondary antibody and DAB staining. This data demonstrates the use of the CFH antibody (Center) for immunohistochemistry. Clinical relevance has not been evaluated.

Confocal immunofluorescent analysis of CFH Antibody (Center) with A549 cell followed by Alexa Fluor 489-conjugated goat anti-rabbit lgG (green). DAPI was used to stain the cell nuclear (blue).

139096

complement factor H

complement factor H

Complement factor H

HF; HF1; HF2  [Similar Products]

CFAH_HUMAN

This gene is a member of the Regulator of Complement Activation (RCA) gene cluster and encodes a protein with twenty short consensus repeat (SCR) domains. This protein is secreted into the bloodstream and has an essential role in the regulation of complement activation, restricting this innate defense mechanism to microbial infections. Mutations in this gene have been associated with hemolytic-uremic syndrome (HUS) and chronic hypocomplementemic nephropathy. Alternate transcriptional splice variants, encoding different isoforms, have been characterized. [provided by RefSeq, Oct 2011]

CFH: Factor H functions as a cofactor in the inactivation of C3b by factor I and also increases the rate of dissociation of the C3bBb complex (C3 convertase) and the (C3b)NBB complex (C5 convertase) in the alternative complement pathway. Genetic variations in CFH are associated with basal laminar drusen (BLD); also known as drusen of Bruch membrane or cuticular drusen or grouped early *****-onset drusen. Drusen are extracellular deposits that accumulate below the retinal pigment epithelium on Bruch membrane. Basal laminar drusen refers to an early *****-onset drusen phenotype that shows a pattern of uniform small, slightly raised yellow subretinal nodules randomly scattered in the macula. In later stages, these drusen often become more numerous, with clustered groups of drusen scattered throughout the retina. In time these small basal laminar drusen may expand and ultimately lead to a serous pigment epithelial detachment of the macula that may result in vision loss. Defects in CFH are the cause of complement factor H deficiency (CFHD). A disorder that can manifest as several different phenotypes, including asymptomatic, recurrent bacterial infections, and renal failure. Laboratory features usually include decreased serum levels of factor H, complement component C3, and a decrease in other terminal complement components, indicating activation of the alternative complement pathway. It is associated with a number of renal diseases with variable clinical presentation and progression, including membranoproliferative glomerulonephritis and atypical hemolytic uremic syndrome. Defects in CFH are a cause of susceptibility to hemolytic uremic syndrome atypical type 1 (AHUS1). 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. 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. Genetic variation in CFH is associated with age-related macular degeneration type 4 (ARMD4). ARMD is a multifactorial eye disease and the most common cause of irreversible vision loss in the developed world. In most patients, the disease is manifest as ophthalmoscopically visible yellowish accumulations of protein and lipid (known as drusen) that lie beneath the retinal pigment epithelium and within an elastin- containing structure known as Bruch membrane. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Secreted, signal peptide; Secreted

Chromosomal Location of Human Ortholog: 1q32

Cellular Component: extracellular space; extracellular region

Molecular Function: heparan sulfate proteoglycan binding; heparin binding; protein binding

Biological Process: complement activation, alternative pathway; regulation of complement activation; innate immune response; complement activation

Disease: Complement Factor H Deficiency; Basal Laminar Drusen; Hemolytic Uremic Syndrome, Atypical, Susceptibility To, 1; Macular Degeneration, Age-related, 4

Dieterich, R., et al. Infect. Immun. 78(11):4467-4476(2010)
Sofat, R., et al. Atherosclerosis 213(1):184-190(2010)
Davila, S., et al. Nat. Genet. 42(9):772-776(2010)
Scambi, C., et al. PLoS ONE 5 (8), E12162 (2010) :
Bunkenborg, J., et al. Proteomics 4(2):454-465(2004)

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