CFHR5 Antibody (PACO63911)
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주문- SKU:
- PACO63911
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
Antibody Name: | CFHR5 Antibody (PACO63911) |
Antibody SKU: | PACO63911 |
Size: | 50ul |
Host Species: | Rabbit |
Tested Applications: | ELISA, WB, IHC |
Recommended Dilutions: | ELISA:1:2000-1:10000, WB:1:500-1:2000, IHC:1:20-1:200 |
Species Reactivity: | Human |
Immunogen: | Recombinant Human Complement factor H-related protein 5 protein (374-569AA) |
Form: | Liquid |
Storage Buffer: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 |
Purification Method: | Antigen Affinity Purified |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
Western Blot. Positive WB detected in: K562 whole cell lysate, HepG2 whole cell lysate, Hela whole cell lysate. All lanes: CFHR5 antibody at 1:1000. Secondary. Goat polyclonal to rabbit IgG at 1/50000 dilution. Predicted band size: 65 kDa. Observed band size: 65 kDa. | |
IHC image of PACO63911 diluted at 1:100 and staining in paraffin-embedded human kidney tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system. |
Background: | Involved in complement regulation. The dimerized forms have avidity for tissue-bound complement fragments and efficiently compete with the physiological complement inhibitor CFH. |
Synonyms: | Complement factor H-related protein 5 (FHR-5), CFHR5, CFHL5 FHR5 |
UniProt Protein Function: | 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. |
UniProt Protein Details: | Protein type:Secreted; Secreted, signal peptide Chromosomal Location of Human Ortholog: 1q31.3 Cellular Component: extracellular region Biological Process: complement activation, alternative pathway Disease: Cfhr5 Deficiency |
NCBI Summary: | 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] |
UniProt Code: | Q9BXR6 |
NCBI GenInfo Identifier: | 13540563 |
NCBI Gene ID: | 81494 |
NCBI Accession: | NP_110414.1 |
UniProt Secondary Accession: | Q9BXR6,Q2NKK2, |
UniProt Related Accession: | Q05CF5,Q5VYL6,Q9BXR6 |
Molecular Weight: | 64,419 Da |
NCBI Full Name: | complement factor H-related protein 5 |
NCBI Synonym Full Names: | complement factor H-related 5 |
NCBI Official Symbol: | CFHR5 |
NCBI Official Synonym Symbols: | FHR5; CFHL5; FHR-5; FLJ10549; MGC133240 |
NCBI Protein Information: | complement factor H-related protein 5; OTTHUMP00000034672 |
UniProt Protein Name: | Complement factor H-related protein 5 |
Protein Family: | Complement factor H-related protein |
UniProt Gene Name: | CFHR5 |
UniProt Entry Name: | FHR5_HUMAN |
Antibodies |
Anti-CFHR5 Antibody (CAB10367) |
Secondary Antibody |
Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014) |
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