产品名称
PIGA Rabbit Polyclonal Antibody
蛋白名称
Phosphatidylinositol N-acetylglucosaminyltransferase subunit A (EC 2.4.1.198) (GlcNAc-PI synthesis protein) (Phosphatidylinositol-glycan biosynthesis class A protein) (PIG-A)
存储缓冲液
Liquid in PBS containing 50% glycerol, and 0.02% New type preservative N.
Human Swissprot No.
P37287
Human Swissprot Link
https://www.uniprot.org/uniprotkb/P37287/entry
Mouse Swissprot No.
Q64323
Mouse Swissprot Link
http://www.uniprot.org/uniprot/Q64323
免疫原
Synthesized peptide derived from human protein . at AA range: 410-490
特异性
PIGA Polyclonal Antibody detects endogenous levels of protein.
稀释度
WB 1:500-2000 ELISA 1:5000-20000
宿主
Polyclonal, Rabbit,IgG
背景介绍
This gene encodes a protein required for synthesis of N-acetylglucosaminyl phosphatidylinositol (GlcNAc-PI), the first intermediate in the biosynthetic pathway of GPI anchor. The GPI anchor is a glycolipid found on many blood cells and which serves to anchor proteins to the cell surface. Paroxysmal nocturnal hemoglobinuria, an acquired hematologic disorder, has been shown to result from mutations in this gene. Alternate splice variants have been characterized. A related pseudogene is located on chromosome 12. [provided by RefSeq, Jun 2010],
细胞定位
Endoplasmic reticulum membrane; Single-pass membrane protein.
信号通路
Glycosylphosphatidylinositol(GPI)-anchor biosynthesis;
功能
catalytic activity:UDP-N-acetyl-D-glucosamine + 1-phosphatidyl-1D-myo-inositol = UDP + 6-(N-acetyl-alpha-D-glucosaminyl)-1-phosphatidyl-1D-myo-inositol.,disease:Defects in PIGA are the cause of paroxysmal nocturnal hemoglobinuria (PNH) [MIM:311770]. PNH is an acquired hemolytic blood disorder characterized by chronic hemolysis with hemoglobinuria, increased tendency to venous thrombosis, and variable degrees of bone marrow failure Biosynthesis of the GPI anchor is deficient in patients with PNH leading to deficient surface expression of GPI-anchored proteins such as DAF or CD59 which play roles in the protection of red cells from the action of complement.,function:Necessary for the synthesis of N-acetylglucosaminyl-phosphatidylinositol, the very early intermediate in GPI-anchor biosynthesis.,online information:Phosphatidylinositol N-acetylglucosaminyltransferase subunit A,pathway:Glycolipid biosynthesis; glycosylphosphatidylinositol-anchor biosynthesis.,similarity:Belongs to the glycosyltransferase 1 family.,subunit:Associates with PIGC, PIGH, PIGP, PIGQ and DPM2. The latter is not essential for activity. Interacts directly with PIGY.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
