Metabolism Antibodies 2
Anti-PIGA Antibody (CAB6236)
- SKU:
- CAB6236
- Product Type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Metabolism
Description
| Antibody Name: | Anti-PIGA Antibody |
| Antibody SKU: | CAB6236 |
| Antibody Size: | 20uL, 50uL, 100uL |
| Application: | WB |
| Reactivity: | Human, Mouse, Rat |
| Host Species: | Rabbit |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 180-300 of human PIGA (NP_002632.1). |
| Application: | WB |
| Recommended Dilution: | WB 1:500 - 1:2000 |
| Reactivity: | Human, Mouse, Rat |
| Positive Samples: |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 180-300 of human PIGA (NP_002632.1). |
| Purification Method: | Affinity purification |
| Storage Buffer: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Isotype: | IgG |
| Sequence: | HIIC VSYT SKEN TVLR AALN PEIV SVIP NAVD PTDF TPDP FRRH DSIT IVVV SRLV YRKG IDLL SGII PELC QKYP DLNF IIGG EGPK RIIL EEVR ERYQ LHDR VRLL GALE HKDV RNVL V |
| Gene ID: | 5277 |
| Uniprot: | P37287 |
| Cellular Location: | Endoplasmic reticulum membrane, Single-pass membrane protein |
| Calculated MW: | 28kDa/35kDa/54kDa |
| Observed MW: | Refer to figures |
| Synonyms: | PIGA, GPI3, MCAHS2, PIG-A, PNH1 |
| Background: | 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. |
| UniProt Protein Function: | PIGA: 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). 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. Defects in PIGA are the cause of multiple congenital anomalies-hypotonia-seizures syndrome type 2 (MCAHS2). An X-linked recessive developmental disorder characterized by dysmorphic features, neonatal hypotonia, myoclonic seizures, and variable congenital anomalies involving the central nervous, cardiac, and urinary systems. Most affected individuals die in infancy. Belongs to the glycosyltransferase group 1 family. Glycosyltransferase 4 subfamily. 3 isoforms of the human protein are produced by alternative splicing. |
| UniProt Protein Details: | Protein type:EC 2.4.1.198; Glycan Metabolism - glycosylphosphatidylinositol (GPI)-anchor biosynthesis; Membrane protein, integral; Transferase Chromosomal Location of Human Ortholog: Xp22.1 Cellular Component: endoplasmic reticulum membrane; glycosylphosphatidylinositol-N-acetylglucosaminyltransferase (GPI-GnT) complex; membrane Molecular Function:phosphatidylinositol N-acetylglucosaminyltransferase activity; protein binding; UDP-glycosyltransferase activity Biological Process: GPI anchor biosynthetic process; positive regulation of metabolic process; preassembly of GPI anchor in ER membrane Disease: Multiple Congenital Anomalies-hypotonia-seizures Syndrome 2; Paroxysmal Nocturnal Hemoglobinuria 1 |
| NCBI Summary: | 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] |
| UniProt Code: | P37287 |
| NCBI GenInfo Identifier: | 585696 |
| NCBI Gene ID: | 5277 |
| NCBI Accession: | P37287.1 |
| UniProt Secondary Accession: | P37287,Q16025, Q16250, B4E0V2, |
| UniProt Related Accession: | P37287 |
| Molecular Weight: | 28,034 Da |
| NCBI Full Name: | Phosphatidylinositol N-acetylglucosaminyltransferase subunit A |
| NCBI Synonym Full Names: | phosphatidylinositol glycan anchor biosynthesis class A |
| NCBI Official Symbol: | PIGA |
| NCBI Official Synonym Symbols: | GPI3; PNH1; PIG-A; MCAHS2 |
| NCBI Protein Information: | phosphatidylinositol N-acetylglucosaminyltransferase subunit A |
| UniProt Protein Name: | Phosphatidylinositol N-acetylglucosaminyltransferase subunit A |
| UniProt Synonym Protein Names: | GlcNAc-PI synthesis protein; Phosphatidylinositol-glycan biosynthesis class A protein; PIG-A |
| Protein Family: | Phosphatidylinositol N-acetylglucosaminyltransferase |
| UniProt Gene Name: | PIGA |
| UniProt Entry Name: | PIGA_HUMAN |
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