Rabbit Anti-PIGA/FITC Conjugated antibody

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GlcNAc PI synthesis protein; GlcNAc-PI synthesis protein; GPI3; Phosphatidylinositol glycan anchor biosynthesis class A; phosphatidylinositol glycan anchor biosynthesis, class A (paroxysmal nocturnal hemoglobinuria); Phosphatidylinositol N-acetylglucosami

Cat:

SL9524R-FITC

Species Reactivity：

(predicted: Human,Mouse,Rat,Chicken,Dog,Pig,Cow,Horse,)

Immunogen：

KLH conjugated synthetic peptide derived from human PIGA

Format：

Lyophilized or Liquid

Storage instructions：

Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of ant

Buffer：

0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Calculated MW：

54kDa

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Unit:

Price: $596.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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background:
PIGA belongs to the glycosyltransferase 1 family and is necessary for the synthesis of N-acetylglucosaminyl-phosphatidylinositol, the very early intermediate in GPI-anchor biosynthesis. Defects in PIGA are the cause of paroxysmal nocturnal hemoglobinuria (PNH) which is an acquired hemolytic blood disorder characterized by chronic hemolysis with hemoglobinuria, increased tendency to venous thrombosis, and variable degrees of bone marrow failure.

Function:
Necessary for the synthesis of N-acetylglucosaminyl-phosphatidylinositol, the very early intermediate in GPI-anchor biosynthesis.

Subunit:
Associates with PIGC, PIGH, PIGP, PIGQ and DPM2. The latter is not essential for activity. Interacts directly with PIGY.

Subcellular Location:
Endoplasmic reticulum membrane; Single-pass membrane protein

DISEASE:
Defects in PIGA are the cause of paroxysmal nocturnal hemoglobinuria (PNH) [MIM:300818]. PNH is a disorder characterized by hemolytic anemia with hemoglobinuria, thromboses in large vessels, and a deficiency in hematopoiesis. Clinical manifestation of red blood cell breakdown with release of hemoglobin into the urine is manifested most prominently by dark-colored urine in the morning.

Similarity:
Belongs to the glycosyltransferase group 1 family. Glycosyltransferase 4 subfamily.

Database links:

Entrez Gene: 5277 Human

Entrez Gene: 18700 Mouse

Omim: 311770 Human

SwissProt: P37287 Human

SwissProt: Q64323 Mouse

Unigene: 137154 Human

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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