PIGV Antibody, Biotin conjugated

Code CSB-PA868321LD01HU
Size US$166
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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) PIGV Polyclonal antibody
Uniprot No.
Target Names
PIGV
Alternative Names
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
Raised in
Rabbit
Species Reactivity
Human
Immunogen
Recombinant Human GPI mannosyltransferase 2 protein (400-469AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
Biotin
Clonality
Polyclonal
Isotype
IgG
Purification Method
>95%, Protein G purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Form
Liquid
Tested Applications
ELISA
Protocols
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
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.
Usage
For Research Use Only. Not for use in diagnostic or therapeutic procedures.

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Target Background

Function
Alpha-1,6-mannosyltransferase involved in glycosylphosphatidylinositol-anchor biosynthesis. Transfers the second mannose to the glycosylphosphatidylinositol during GPI precursor assembly.
Gene References into Functions
  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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Involvement in disease
Hyperphosphatasia with mental retardation syndrome 1 (HPMRS1)
Subcellular Location
Endoplasmic reticulum membrane; Multi-pass membrane protein.
Protein Families
PIGV family
Database Links

HGNC: 26031

OMIM: 239300

KEGG: hsa:55650

STRING: 9606.ENSP00000078527

UniGene: Hs.259605

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