GNS Antibody
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中文名稱：GNS兔多克隆抗體
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貨號：CSB-PA009639ESR1HU
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規格：￥440
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圖片：
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Immunohistochemistry of paraffin-embedded human testis tissue using CSB-PA009639ESR1HU at dilution of 1:100 -
Immunohistochemistry of paraffin-embedded human kidney tissue using CSB-PA009639ESR1HU at dilution of 1:100
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其他：
產品詳情
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產品名稱：Rabbit anti-Homo sapiens (Human) GNS Polyclonal antibody
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Uniprot No.：
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基因名：GNS
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別名：2610016K11Rik antibody; AU042285 antibody; C87209 antibody; G6S antibody; Glucosamine (N-acetyl) 6 sulfatase antibody; Glucosamine 6 sulfatase antibody; Glucosamine-6-sulfatase antibody; GNS antibody; GNS_HUMAN antibody; MGC21274 antibody; N acetylglucosamine 6 sulfatase [Precursor] antibody; N-acetylglucosamine-6-sulfatase antibody; N28088 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 N-acetylglucosamine-6-sulfatase protein (293-552AA)
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免疫原種屬：Homo sapiens (Human)
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標記方式：Non-conjugated
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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：PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
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產品提供形式：Liquid
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應用范圍：ELISA, IHC
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推薦稀釋比：
Application Recommended Dilution IHC 1:20-1:200 -
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.
產品評價
相關產品
靶點詳情
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基因功能參考文獻：
- Mice deficient in GNS showed lysosomal storage pathology and a phenotype that closely resembled human MPSIIID. Moreover, treatment of the GNS-deficient animals with GNS-encoding adeno-associated viral (AAV) vectors of serotype 9 delivered to the cerebrospinal fluid completely corrected pathological storage, improved lysosomal functionality in the CNS and somatic tissues, resolved neuroinflammation PMID: 28334745
- 12 new patients and 15 novel mutations were identified in Mucopolysaccharidosis type IIID. PMID: 20232353
- The Sanfilippo syndrome type D patient was found to be homozygous for a single base pair deletion (c1169delA), which will cause a frameshift and premature termination of N-acetylglucosamine-6-sulphatase. PMID: 12624138
- A large intragenic deletion of 8723 bp encompassing exons 2 and 3 has been identified, the first large intragenic deletion to be reported in any of the four Sanfilippo subtypes. Q272X has also been found. PMID: 16990043
- Sanfilippo syndrome type D has 3 novel mutations in the GNS Gene. PMID: 17998446
- We identified the novel homozygous single base pair insertion, c.1226GinsG, which leads to a frame-shift and a premature truncation of the GNS protein (p.R409Rfs21X). PMID: 19650410
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相關疾病：Mucopolysaccharidosis 3D (MPS3D)
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亞細胞定位：Lysosome.
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蛋白家族：Sulfatase family
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數據庫鏈接：
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