Factor H Active Protein | CFH active protein

Factor H Protein, Human, Recombinant (His Tag)

Cat. Num. AAA8120449

• Gene Names: CFH; FH; HF; HF1; HF2; HUS; FHL1; AHUS1; AMBP1; ARMD4; ARMS1; CFHL3
• Purity: >95% as determined by SDS-PAGE
• Synonyms: Factor H; Factor H Protein; Human; Recombinant (His Tag); Human Complement Factor H/CFH Protein (His Tag); AHUS1 Protein; AMBP1 Protein; ARMD4 Protein; ARMS1 Protein; CFHL3 Protein; FH Protein; FHL1 Protein; HF Protein; HF1 Protein; HF2 Protein; HUS Protein; complement factor H; CFH active protein

• Host: HEK293 Cells
• Purity/Purification: >95% as determined by SDS-PAGE
• Form/Format: Lyophilized from sterile PBS, pH7.4. Normally 5%-8% trehalose, mannitol and 0.01% Tween80 are added as protectants before lyophilization. Please refer to the specific buffer information in the hard copy of CoA.
• Sequence: Ser860-Arg1231

• Species: Human
• Activity: Measured by its ability to bind biotinylated human DMP1 in a functional ELISA.
• Endotoxin: <1.0EU per ug of the protein as determined by the LAL method
• Predicted N Terminal: Ser 860
• Tag: C-His
• Protein Construction: A DNA sequence encoding the C-terminal segment of CFH isoform a (NP_000177.2), corresponding to amino acid (Ser 860-Arg 1231) was expressed, fused with a polyhistidine tag at the C-terminus and a signal peptide at the N-terminus.
• Reconstitution: A hardcopy of COA with reconstitution instruction is sent along with the products. Please refer to it for detailed information.
• Preparation and Storage: Samples are stable for up to twelve months from date of receipt at -20 degree C to -80 degree C. Store it under sterile conditions at -20 degree C to -80 degree C. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.; In general, recombinant proteins are provided as lyophilized powder which are shipped at ambient temperature. Bulk packages of recombinant proteins are provided as frozen liquid.; They are shipped out with blue ice.

SDS-Page

Testing Data

(Measured by its ability to bind biotinylated human DMP1 in a functional ELISA.)

Related Product Information for CFH active protein

Background: Complement factor H, also known as H factor 1, and CFH, is a sialic acid containing glycoprotein that plays an integral role in the regulation of the complement-mediated immune system that is involved in microbial defense, immune complex processing, and programmed cell death. Factor H protects host cells from injury resulting from unrestrained complement activation. CFH regulates complement activation on self cells by possessing both cofactor activity for the Factor I mediated C3b cleavage, and decay accelerating activity against the alternative pathway C3 convertase, C3bBb. CFH protects self cells from complement activation but not bacteria/viruses. Due to the central role that CFH plays in the regulation of complement, there are many clinical implications arrising from aberrant CFH activity. Mutations in the Factor H gene are associated with severe and diverse diseases including the rare renal disorders hemolytic uremic syndrome (HUS) and membranoproliferative glomerulonephritis (MPGN) also termed dense deposit disease (DDD), membranoproliferative glomuleronephritis type II or dense deposit disease, as well as the more frequent retinal disease age related macular degeneration (AMD). In addition to its complement regulatory activities, factor H has multiple physiological activities and 1) acts as an extracellular matrix component, 2) binds to cellular receptors of the integrin type, and 3) interacts with a wide selection of ligands, such as the C-reactive protein, thrombospondin, bone sialoprotein, osteopontin, and heparin.

Product Categories/Family for CFH active protein

Complement Activation Pathways

References

Zipfel PF. (2001) Complement factor H: physiology and pathophysiology. Semin Thromb Hemost. 27(3): 191-9.Zipfel PF, et al. (2008) The complement fitness factor H: role in human diseases and for immune escape of pathogens, like pneumococci. Vaccine. 26 Suppl 8: I67-74.Ferreira VP, et al. (2010) Complement control protein factor H: the good, the bad, and the inadequate. Mol Immunol. 47(13): 2187-97.Donoso LA, et al. (2010) The role of complement Factor H in age-related macular degeneration: a review. Surv Ophthalmol. 55(3): 227-46.

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