GNS Antibody
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中文名稱:GNS兔多克隆抗體
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貨號:CSB-PA009639GA01HU
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規(guī)格:¥3,900
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其他:
產(chǎn)品詳情
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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,Mouse,Rat
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免疫原:Human GNS
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免疫原種屬:Homo sapiens (Human)
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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 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
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產(chǎn)品提供形式:Liquid
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應用范圍:ELISA,WB,IHC
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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.
相關(guān)產(chǎn)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(guān)疾?。?/div>Mucopolysaccharidosis 3D (MPS3D)亞細胞定位:Lysosome.蛋白家族:Sulfatase family數(shù)據(jù)庫鏈接:
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