CFHR5 Antibody

中文名稱：

CFHR5兔多克隆抗體
貨號：

CSB-PA883624LA01HU
規(guī)格：

￥440
圖片：
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 CSB-PA883624LA01HU 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.
其他：

產(chǎn)品詳情
產(chǎn)品評價
相關(guān)產(chǎn)品
靶點詳情

產(chǎn)品詳情

產(chǎn)品名稱：

Rabbit anti-Homo sapiens (Human) CFHR5 Polyclonal antibody
Uniprot No.：

Q9BXR6
基因名：

CFHR5
別名：

CFHL5 antibody; CFHR5 antibody; CFHR5D antibody; Complement factor H-related 5 antibody; Complement factor H-related protein 5 antibody; factor H-related gene 5 antibody; factor H-related protein 5 antibody; FHR-5 antibody; FHR5 antibody; FHR5_HUMAN antibody; FLJ10549 antibody; MGC133240 antibody; OTTHUMP00000034672 antibody
宿主：

Rabbit
反應(yīng)種屬：

Human
免疫原：

Recombinant Human Complement factor H-related protein 5 protein (374-569AA)
免疫原種屬：

Homo sapiens (Human)

標記方式：

Non-conjugated

本頁面中的產(chǎn)品，CFHR5 Antibody (CSB-PA883624LA01HU)，的標記方式是Non-conjugated。對于CFHR5 Antibody，我們還提供其他標記。見下表：

標記方式	貨號	產(chǎn)品名稱	應(yīng)用
HRP	CSB-PA883624LB01HU	CFHR5 Antibody, HRP conjugated	ELISA
FITC	CSB-PA883624LC01HU	CFHR5 Antibody, FITC conjugated
Biotin	CSB-PA883624LD01HU	CFHR5 Antibody, Biotin conjugated	ELISA

克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

Antigen Affinity Purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
產(chǎn)品提供形式：

Liquid
應(yīng)用范圍：

ELISA, WB, IHC

推薦稀釋比：

Application	Recommended Dilution
WB	1:500-1:2000
IHC	1:20-1:200

Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
Immunohistochemistry (IHC) Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產(chǎn)品評價

靶點詳情

功能：

Involved in complement regulation. The dimerized forms have avidity for tissue-bound complement fragments and efficiently compete with the physiological complement inhibitor CFH.
基因功能參考文獻：
1. Novel genetic rearrangement generated from a heterozygous deletion spanning 146 Kbp involving multiple CFHR genes leading to a CFHR1-R5 hybrid protein. This deletion was found in four family members presenting with a familial dominant glomerulopathy. PMID: 28729035
2. Higher serum FHR-5 levels were associated with a lack of response to immunosuppression, the presence of endocapillary hypercellularity, and histology scores of IgA nephropathy severity. PMID: 28673452
3. Our study found that rare variants in CFHR5 may contribute to the genetic susceptibility to IgA Nephropathy, which suggests that CFHR5 is an IgA Nephropathy susceptibility gene PMID: 26825529
4. Next-generation sequencing of the CFH region identified putatively functional variants (missense, splice site and indel) on the four common haplotypes. We found no expression of any of the five CFH-related genes in the retina or RPE/Choroid/Sclera, in contrast to the liver, which is the main source of the circulating proteins. [CFHR5] PMID: 27196323
5. Studies indicate that complement factor H-related proteins (FHR1-5) may enhance complement activation, with important implications for the role of these proteins in disease. PMID: 25979655
6. In this study, we identify pentraxin 3 (PTX3) as a novel ligand of CFHR5 PMID: 25855355
7. At least two distinct intronic breakpoints within the CFHR5 gene can cause the same mutant CFHR5 protein and C3 glomerulopathy. PMID: 24067434
8. A hybrid CFHR2-CFHR5 plasma protein, arising from a chromosomal deletion mutation stabilizes the C3 convertase and reduces factor H-mediated convertase decay. PMID: 24334459
9. Recent investigations in London and Cyprus culminated in the identification of another autosomal dominant condition that presents with microscopic haematuria because of heterozygous mutations in the CFHR5 gene--{review} PMID: 23402027
10. A potentially pathogenic sequence variation was found in CFHR5 in the patients with atypical hemolytic uremic syndrome. PMID: 22622361
11. CFHR5 nephropathy is discussed. PMID: 22065842
12. Describe the clinical course, significant variable expressivity, and marked gender difference regarding the development of chronic renal failure in familial C3 glomerulopathy associated with CFHR5 mutations. PMID: 21566112
13. evidence for an inherited renal disease, endemic in Cyprus, characterised by microscopic and synpharyngitic macroscopic haematuria, renal failure and C3 glomerulonephritis; affected individuals have an internal duplication within the gene for CFHR5 PMID: 20800271
14. Study identified novel mutations in CFH, CFHR5, CFI, CFB and C3 in American patients with atypical hemolytic uremic syndrome. PMID: 20513133
15. Maps to between FHR-2 and the non-complement protein factor XIIIb at 1q32. PMID: 12041828
16. FHR-5 shares properties of binding heparin and C-reactive protein and lipoprotein association with one or more of the other FHRs, but is unique among this family of proteins in possessing independent complement-regulatory activity. PMID: 15879123
17. Identification of specific variants of variants of CFHR5 in membranoproliferative glomerulonephritis type II. PMID: 16299065
18. CFHR5 genetic alterations may play a secondary role in the pathogenesis of haemolytic uraemic syndrome. PMID: 17000000
19. No definitive pathogenic CFHR5 mutations have been found in any of 639 unrelated patients with age-related macular degeneration (AMD), indicating that sequence variations in CFHR5 do not play a major role in determining AMD susceptibility. PMID: 19365580
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相關(guān)疾?。?/div>
CFHR5 deficiency (CFHR5D)
亞細胞定位：

Secreted.
組織特異性：

Expressed by the liver and secreted in plasma.
數(shù)據(jù)庫鏈接：

HGNC: 24668

OMIM: 608593

KEGG: hsa:81494

STRING: 9606.ENSP00000256785

UniGene: Hs.282594

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