PIGV Antibody
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货号:CSB-PA868321LA01HU
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规格:¥440
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促销:
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图片:
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Western Blot
Positive WB detected in: A549 whole cell lysate
All lanes: PIGV antibody at 4.6µg/ml
Secondary
Goat polyclonal to rabbit IgG at 1/50000 dilution
Predicted band size: 56 kDa
Observed band size: 56 kDa -
IHC image of CSB-PA868321LA01HU diluted at 1:200 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.
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Immunofluorescence staining of Hela cells with CSB-PA868321LA01HU at 1:66, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
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其他:
产品详情
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产品名称:Rabbit anti-Homo sapiens (Human) PIGV Polyclonal antibody
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Uniprot No.:Q9NUD9
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基因名:PIGV
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别名:GPI mannosyltransferase 2 antibody; GPI mannosyltransferase II antibody; GPI MT II antibody; GPI-MT-II antibody; Phosphatidylinositol glycan biosynthesis class V protein antibody; Phosphatidylinositol-glycan biosynthesis class V protein antibody; PIG-V antibody; Pigv antibody; PIGV_HUMAN antibody
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宿主:Rabbit
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反应种属:Human
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免疫原:Recombinant Human GPI mannosyltransferase 2 protein (400-469AA)
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免疫原种属:Homo sapiens (Human)
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标记方式:Non-conjugated
本页面中的产品,PIGV Antibody (CSB-PA868321LA01HU),的标记方式是Non-conjugated。对于PIGV Antibody,我们还提供其他标记。见下表:
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克隆类型:Polyclonal
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抗体亚型:IgG
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纯化方式:>95%, Protein G purified
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浓度:It differs from different batches. Please contact us to confirm it.
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保存缓冲液:Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 -
产品提供形式:Liquid
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应用范围:ELISA, WB, IHC, IF
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推荐稀释比:
Application Recommended Dilution WB 1:500-1:5000 IHC 1:200-1:500 IF 1:50-1:200 -
Protocols:
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储存条件:Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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货期: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.
相关产品
靶点详情
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功能:Alpha-1,6-mannosyltransferase involved in glycosylphosphatidylinositol-anchor biosynthesis. Transfers the second mannose to the glycosylphosphatidylinositol during GPI precursor assembly.
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基因功能参考文献:
- Data indicate that mannosyltransferases PIGV mutations are the major cause of hyperphosphatasia-mental retardation syndrome (HPMRS) which displays a broad clinical variability regarding associated malformations and growth patterns. PMID: 24129430
- PIGV is the rate-limiting enzyme in GPI biosynthesis under limited dolicholphosphate mannose availability. PMID: 23694781
- Hyperphosphatasia resulted from secretion of ALP, a GPI-anchored protein normally expressed on the cell surface, into serum due to PIGV deficiency. PMID: 22228761
- novel compound heterozygous mutations in the PIGV gene c.467G>A and c.1022C>A and a homozygous mutation c.1022C>A in hyperphosphatasia-mental retardation syndrome PMID: 21739589
- PIGV mutations are associated with hyperphosphatasia mental retardation syndrome. PMID: 20802478
- PIG-V is the second mannosyltransferase in GPI anchor biosynthesis. PMID: 15623507
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相关疾病:Hyperphosphatasia with mental retardation syndrome 1 (HPMRS1)
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亚细胞定位:Endoplasmic reticulum membrane; Multi-pass membrane protein.
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蛋白家族:PIGV family
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数据库链接:
HGNC: 26031
OMIM: 239300
KEGG: hsa:55650
STRING: 9606.ENSP00000078527
UniGene: Hs.259605
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