Rabbit anti-Human CFH Polyclonal Antibody | anti-CFH antibody

CFH, ID (CFH, HF, HF1, HF2, Complement factor H, H factor 1) (AP)

Cat. Num. AAA6285348

• Gene Names: CFH; FH; HF; HF1; HF2; HUS; FHL1; AHUS1; AMBP1; ARMD4; ARMS1; CFHL3
• Reactivity: Human
• Applications: Immunohistochemistry, ELISA, Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: CFH; Polyclonal Antibody; ID (CFH; HF; HF1; HF2; Complement factor H; H factor 1) (AP); H factor 1; anti-CFH antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Alkaline Phosphatase (AP).

• Applicable Applications for anti-CFH antibody: Immunohistochemistry (IHC), ELISA (EIA), Western Blot (WB)
• Application Notes: IHC: 1:50-100; ELISA: 1:1,000; WB: 1:100-500; Applications are based on unconjugated antibody.
• Immunogen: 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.
• Conjugate: AP
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

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

Immunohistochemistry (IHC)

(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.)

Immunofluorescence (IF)

(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).)

Related Product Information for anti-CFH antibody

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.

Product Categories/Family for anti-CFH antibody

Antibodies; Complement

References

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).

NCBI and Uniprot Product Information

• NCBI GI #: 62739186
• NCBI GeneID: 3075
• NCBI Accession #: NP_000177.2
• NCBI GenBank Nucleotide #: NM_000186.3
• UniProt Accession #: P08603; P78435; Q14570; Q2TAZ5; Q38G77; Q5TFM3; Q8N708; Q9NU86; A5PL14
• Molecular Weight: 1231 (449)
• NCBI Official Full Name: complement factor H isoform a
• NCBI Official Synonym Full Names: complement factor H
• NCBI Official Symbol: CFH
• NCBI Official Synonym Symbols: FH; HF; HF1; HF2; HUS; FHL1; AHUS1; AMBP1; ARMD4; ARMS1; CFHL3
• NCBI Protein Information: complement factor H; beta-1H; factor H-like 1; beta-1-H-globulin; H factor 1 (complement); H factor 2 (complement); adrenomedullin binding protein; complement factor H, isoform b; age-related maculopathy susceptibility 1
• UniProt Protein Name: Complement factor H
• Protein Family: Complement factor
• UniProt Gene Name: CFH
• UniProt Synonym Gene Names: HF; HF1; HF2
• UniProt Entry Name: CFAH_HUMAN

Uniprot Description

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 adult-onset drusen. Drusen are extracellular deposits that accumulate below the retinal pigment epithelium on Bruch membrane. Basal laminar drusen refers to an early adult-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; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 1q32

Cellular Component: extracellular space; extracellular region

Molecular Function: heparin binding; heparan sulfate proteoglycan 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