Western Blot (WB)
(Staining (0. 03ug/ml) of human lung lysate (RIPA buffer, 35ug total protein per lane). Primary incubated for 1 hour. Detected by western blot using chemiluminescence.)
Goat Complement factor H Polyclonal Antibody | anti-CFH antibody
Goat anti-Complement factor H Antibody
Western Blot: Approx 170kDa band observed in human lung lysates (calculated MW of 139kDa according to NP_000177.2). Recommended concentration: 0. 03-0.1ug/ml.
Western Blot (WB)
(Staining (0. 03ug/ml) of human lung lysate (RIPA buffer, 35ug total protein per lane). Primary incubated for 1 hour. Detected by western blot using chemiluminescence.)
Western Blot (WB)
(Staining (0. 03ug/ml) of human lung lysate (RIPA buffer, 35ug total protein per lane). Primary incubated for 1 hour. Detected by western blot using chemiluminescence.)
NCBI and Uniprot Product Information
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 region; extracellular space
Molecular Function: heparan sulfate proteoglycan binding; heparin binding; protein binding
Biological Process: complement activation; complement activation, alternative pathway; regulation of complement activation
Disease: Basal Laminar Drusen; Complement Factor H Deficiency; Hemolytic Uremic Syndrome, Atypical, Susceptibility To, 1; Macular Degeneration, Age-related, 4
Research Articles on CFH
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Product Notes
The CFH cfh (Catalog #AAA420873) is an Antibody produced from Goat and is intended for research purposes only. The product is available for immediate purchase. The Goat anti-Complement factor H Antibody reacts with Tested: Human; Expected from sequence similarity: Human and may cross-react with other species as described in the data sheet. AAA Biotech's Complement factor H can be used in a range of immunoassay formats including, but not limited to, Peptide ELISA (EIA), Western Blot (WB). Peptide ELISA: Antibody detection limit dilution 1: 32000. Western Blot: Approx 170kDa band observed in human lung lysates (calculated MW of 139kDa according to NP_000177.2). Recommended concentration: 0. 03-0.1ug/ml. Researchers should empirically determine the suitability of the CFH cfh for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. The amino acid sequence is listed below: HLVPDRKKDQ YK. It is sometimes possible for the material contained within the vial of "Complement factor H, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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