Mouse Factor H Monoclonal Antibody | anti-CFH antibody

Factor H, Mouse, mAb 1A2

Cat. Num. AAA584435

• Gene Names: CFH; FH; HF; HF1; HF2; HUS; FHL1; AHUS1; AMBP1; ARMD4; ARMS1; CFHL3
• Reactivity: Reactivity: Mouse. Cross reactivity: Rat
• Applications: Western Blot
• Synonyms: Factor H; Monoclonal Antibody; Mouse; mAb 1A2; anti-CFH antibody

• Host: Mouse
• Reactivity: Reactivity: Mouse. Cross reactivity: Rat
• Clonality: Monoclonal
• Isotype: Mouse IgG2b
• Form/Format: 1 ml (100 ug/ml) 0.2 um filtered antibody solution in PBS, containing 0.1% bovine serum albumin and 0.02% sodium azide.; For unit size 0.5 mg or larger: PBS only (concentration< 0.5mg/ml)
• Sequence Length: 185

• Applicable Applications for anti-CFH antibody: F, Immunoasssay (IA), Western Blot
• Application Notes: Application Use: For immunohistology and Western blotting, dilutions to be used depend on detection system applied. It is recommended that users test the reagent and determine their own optimal dilutions. The typical starting working dilution is 1:50.
• Immunogen: Mouse factor H-human IgG fusion protein
• Preparation and Storage: Product should be stored at 4 degree C. Under recommended storage conditions, product is stable for one year.

Related Product Information for anti-CFH antibody

Product Description: Monoclonal antibody 1A2 recognizes mouse complement factor H (CFH). CFH is the first regulatory protein of the alternative pathway of the complement system. There are three pathways of complement activation; classical, alternative, and lectin activation pathway. These pathways converge to form C3/C5 convertases that generate C3a, C3b,C5a, and C5b, each with substantial biologic activity. Complement regulators are necessary to prevent the injudicious production of these mediators and potential injury to self-tissue. The plasma proteins CFH, C4-binding protein and the cell membrane proteins complement receptor 1 (CR1; CD35), decay-accelerating factor (CD55), and membrane co-factor protein (CD46) all are members of the regulators of complement activation family. These proteins have natural affinity for C3b and/or C4b, which confers on them the ability to accelerate the intrinsic decay of C3/C5 convertases and/or act as co-factor for the cleavage and inactivation (i) of C3b and C4b by complement factor I (CFI). Genetic human analyses reveals a clear association of CFH with different human diseases. These include diseases of the kidney, the atypical form of Hemolytic Uremic Syndrome (aHUS) and membranoproliferative glomerulonephritis (MPGN). Furthermore, CFH is associated with age-related macular degeneration (AMD), a disease of the eye.

NCBI and Uniprot Product Information

• NCBI GI #: 183767
• NCBI GeneID: 3075
• Molecular Weight: 51,034 Da
• NCBI Official Full Name: factor H, partial
• 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

NCBI Description

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]

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