Complement Factor H-Related Protein 5 Active Protein | CFHR5 active protein

Complement Factor H-Related Protein 5, Recombinant, Human (FHR5)

Cat. Num. AAA638289

• Gene Names: CFHR5; FHR5; CFHL5; FHR-5; CFHR5D
• Purity: Highly Purified; 90%, as determined by SDS-PAGE and visualized by silver stain.
• Synonyms: Complement Factor H-Related Protein 5; Recombinant; Human (FHR5); CFHR5 active protein

• Purity/Purification: Highly Purified; 90%, as determined by SDS-PAGE and visualized by silver stain.
• Form/Format: Supplied as a lyophilized powder in 25mM Tris, 0.4M sodium chloride, pH 7.5.

• Activity: Measured by its ability to inhibit the binding of biotinylated rhCFHR5 to immobilized rhCRP (McRae, JL., et al., 2005, J. Immunol. 174:6250).
• Endotoxin Level: < 1.0 EU per 1 g of the protein as determined by the LAL method.
• Reconstitution: It is recommended that sterile PBS be added to the vial to prepare a working stock solution of no less than 100ug/ml. The carrier-free protein should be used immediately upon reconstitution to avoid losses in activity due to non-specific binding to the inside surface of the vial. For long term storage as a dilute solution, a carrier protein (e.g. 0.1% HSA or BSA) should be added to the vial.
• Preparation and Storage: Samples are stable for up to twelve months at -20 degree C in a manual defrost freezer after the date of receipt. After reconstitution, this protein can be aliquoted and stored at above temperature for up to three months without a significant loss of activity. Avoid repeated freeze-thaw cycles.

Related Product Information for CFHR5 active protein

The human complement factor H protein family consists of the complement and immune regulators factor H, the factor H-like protein 1(FHL-1) and five factor H-related proteins (FHR-1 to -5) (2). Members of this family are exclusively composed of individually folded protein domains, termed short consensus repeats (SCRs) or complement control modules. The genes of this family have been located in human chromosome 1q32, which is known as the regulators of complement activation (RCA) gene clusters (3). FHR-5 has been identified initially as a universal component of complement deposits (1), and detected in glomerular immune deposits (4). The pattern of deposits is similar to other complement components, suggesting that FHR-5 may play a role in complement activation and regulation. It is synthesized in the liver and consists of 9 SCRs. Its biological function is not understood fully. FHR-5 exhibits similar characteristics as those of factor H in heparin binding, CRP binding, and lipoprotein association (5). Weak factor I-dependent cofactor activity for C3b cleavage has also been observed (5).

Product Categories/Family for CFHR5 active protein

Molecular Biology; MB-Complement

NCBI and Uniprot Product Information

• NCBI GI #: 13540563
• NCBI GeneID: 81494
• NCBI Accession #: NP_110414.1
• NCBI GenBank Nucleotide #: NM_030787.3
• UniProt Accession #: Q9BXR6; Q2NKK2
• Molecular Weight: The purified, secreted rhCFHR5 has a major N-terminal sequence of E19GTLCDFPKI. The 561 amino acid residue rhCFHR5 predicts a molecular mass of 64 kD and migrates with an apparent molecular mass of approximately 73kD in SDS-PAGE under reducing conditions.
• NCBI Official Full Name: complement factor H-related protein 5
• NCBI Official Synonym Full Names: complement factor H-related 5
• NCBI Official Symbol: CFHR5
• NCBI Official Synonym Symbols: FHR5; CFHL5; FHR-5; CFHR5D
• NCBI Protein Information: complement factor H-related protein 5
• UniProt Protein Name: Complement factor H-related protein 5
• Protein Family: Complement factor H-related protein
• UniProt Gene Name: CFHR5
• UniProt Synonym Gene Names: CFHL5; FHR5; FHR-5
• UniProt Entry Name: FHR5_HUMAN

NCBI Description

This gene is a member of a small complement factor H (CFH) gene cluster on chromosome 1. Each member of this gene family contains multiple short consensus repeats (SCRs) typical of regulators of complement activation. The protein encoded by this gene has nine SCRs with the first two repeats having heparin binding properties, a region within repeats 5-7 having heparin binding and C reactive protein binding properties, and the C-terminal repeats being similar to a complement component 3 b (C3b) binding domain. This protein co-localizes with C3, binds C3b in a dose-dependent manner, and is recruited to tissues damaged by C-reactive protein. Allelic variations in this gene have been associated, but not causally linked, with two different forms of kidney disease: membranoproliferative glomerulonephritis type II (MPGNII) and hemolytic uraemic syndrome (HUS). [provided by RefSeq, Jan 2010]

Uniprot Description

CFHR5: Involved in complement regulation. Defects in CFHR5 have been found in patients with atypical hemolytic uremic syndrome and may contribute to the disease. Atypical hemolytic uremic syndrome 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.

Protein type: Secreted, signal peptide; Secreted

Chromosomal Location of Human Ortholog: 1q31.3

Cellular Component: extracellular region

Biological Process: complement activation, alternative pathway

Disease: Cfhr5 Deficiency