PIGV Antibody, Biotin conjugated

說明書

產品詳情

產品名稱：

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

Q9NUD9
基因名：

PIGV
別名：

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
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human GPI mannosyltransferase 2 protein (400-469AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

Biotin
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

>95%, Protein G 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
產品提供形式：

Liquid
應用范圍：

ELISA
Protocols：

ELISA 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.

功能：

Alpha-1,6-mannosyltransferase involved in glycosylphosphatidylinositol-anchor biosynthesis. Transfers the second mannose to the glycosylphosphatidylinositol during GPI precursor assembly.
基因功能參考文獻：
1. 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
2. PIGV is the rate-limiting enzyme in GPI biosynthesis under limited dolicholphosphate mannose availability. PMID: 23694781
3. Hyperphosphatasia resulted from secretion of ALP, a GPI-anchored protein normally expressed on the cell surface, into serum due to PIGV deficiency. PMID: 22228761
4. 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
5. PIGV mutations are associated with hyperphosphatasia mental retardation syndrome. PMID: 20802478
6. PIG-V is the second mannosyltransferase in GPI anchor biosynthesis. PMID: 15623507
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相關疾病：

Hyperphosphatasia with mental retardation syndrome 1 (HPMRS1)
亞細胞定位：

Endoplasmic reticulum membrane; Multi-pass membrane protein.
蛋白家族：

PIGV family
數據庫鏈接：

HGNC: 26031

OMIM: 239300

KEGG: hsa:55650

STRING: 9606.ENSP00000078527

UniGene: Hs.259605

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