CFHR5 Antibody, Biotin conjugated
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中文名稱：CFHR5兔多克隆抗體, Biotin偶聯
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貨號：CSB-PA883624LD01HU
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規格：￥880
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其他：
產品詳情
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產品名稱：Rabbit anti-Homo sapiens (Human) CFHR5 Polyclonal antibody
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Uniprot No.：
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基因名：CFHR5
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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
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宿主：Rabbit
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反應種屬：Human
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免疫原：Recombinant Human Complement factor H-related protein 5 protein (374-569AA)
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免疫原種屬：Homo sapiens (Human)
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標記方式：Biotin
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克隆類型：Polyclonal
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抗體亞型：IgG
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純化方式：Antigen Affinity Purified
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濃度：It differs from different batches. Please contact us to confirm it.
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保存緩沖液：Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 -
產品提供形式：Liquid
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應用范圍：ELISA
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Protocols：
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儲存條件：Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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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.
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用途：For Research Use Only. Not for use in diagnostic or therapeutic procedures.
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靶點詳情
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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.
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基因功能參考文獻：
- 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
- 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
- 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
- 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
- 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
- In this study, we identify pentraxin 3 (PTX3) as a novel ligand of CFHR5 PMID: 25855355
- At least two distinct intronic breakpoints within the CFHR5 gene can cause the same mutant CFHR5 protein and C3 glomerulopathy. PMID: 24067434
- 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
- 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
- A potentially pathogenic sequence variation was found in CFHR5 in the patients with atypical hemolytic uremic syndrome. PMID: 22622361
- CFHR5 nephropathy is discussed. PMID: 22065842
- 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
- 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
- Study identified novel mutations in CFH, CFHR5, CFI, CFB and C3 in American patients with atypical hemolytic uremic syndrome. PMID: 20513133
- Maps to between FHR-2 and the non-complement protein factor XIIIb at 1q32. PMID: 12041828
- 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
- Identification of specific variants of variants of CFHR5 in membranoproliferative glomerulonephritis type II. PMID: 16299065
- CFHR5 genetic alterations may play a secondary role in the pathogenesis of haemolytic uraemic syndrome. PMID: 17000000
- 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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相關疾病：CFHR5 deficiency (CFHR5D)
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亞細胞定位：Secreted.
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組織特異性：Expressed by the liver and secreted in plasma.
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數據庫鏈接：
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