The PIGN Polyclonal Antibody (PACO60781) is a valuable tool for researchers studying the PIGN protein, which is involved in glycosylphosphatidylinositol (GPI) anchor biosynthesis. This antibody, generated in rabbits, exhibits high reactivity with human samples and is validated for use in Western blot applications. By binding to the PIGN protein, this antibody enables accurate detection and analysis in a variety of cell types, making it ideal for investigations in glycobiology and genetic disorders.
PIGN is a critical component in the GPI anchor biosynthesis pathway, which is essential for anchoring proteins to the cell membrane. Dysregulation of PIGN has been linked to various genetic disorders, including congenital disorders of glycosylation, making it a promising target for further research in these areas. Understanding the role of PIGN in GPI anchor biosynthesis is crucial for unraveling the pathophysiology of these disorders and developing potential therapeutic interventions.
Antibody Name:
PIGN Antibody (PACO60781)
Antibody SKU:
PACO60781
Size:
50ug
Host Species:
Rabbit
Tested Applications:
ELISA, IHC
Recommended Dilutions:
ELISA:1:2000-1:10000, IHC:1:200-1:500
Species Reactivity:
Human
Immunogen:
Recombinant Human GPI ethanolamine phosphate transferase 1 protein (27-181AA)
IHC image of PACO60781 diluted at 1:200 and staining in paraffin-embedded human endometrial cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
Background:
Ethanolamine phosphate transferase involved in glycosylphosphatidylinositol-anchor biosynthesis. Transfers ethanolamine phosphate to the first alpha-1,4-linked mannose of the glycosylphosphatidylinositol precursor of GPI-anchor (By similarity). May act as suppressor of replication stress and chromosome missegregation.
Synonyms:
GPI ethanolamine phosphate transferase 1 (EC 2) (MCD4 homolog) (Phosphatidylinositol-glycan biosynthesis class N protein) (PIG-N), PIGN, MCD4
UniProt Protein Function:
PIGN: Ethanolamine phosphate transferase involved in glycosylphosphatidylinositol-anchor biosynthesis. Transfers ethanolamine phosphate to the first alpha-1,4-linked mannose of the glycosylphosphatidylinositol precursor of GPI-anchor. Defects in PIGN are the cause of multiple congenital anomalies-hypotonia-seizures syndrome type 1 (MCAHS1). An autosomal recessive disorder characterized by neonatal hypotonia, lack of psychomotor development, seizures, dysmorphic features, and variable congenital anomalies involving the cardiac, urinary, and gastrointestinal systems. Most affected individuals die before 3 years of age. Belongs to the PIGG/PIGN/PIGO family. PIGN subfamily.Protein type: EC 2.-.-.-; Membrane protein, multi-pass; Transferase; Membrane protein, integral; Glycan Metabolism - glycosylphosphatidylinositol (GPI)-anchor biosynthesis; Endoplasmic reticulumChromosomal Location of Human Ortholog: 18q21.33Cellular Component: endoplasmic reticulum membrane; membraneMolecular Function: mannose-ethanolamine phosphotransferase activity; phosphotransferase activity, for other substituted phosphate groupsBiological Process: GPI anchor biosynthetic process; preassembly of GPI anchor in ER membraneDisease: Multiple Congenital Anomalies-hypotonia-seizures Syndrome 1
UniProt Protein Details:
NCBI Summary:
This gene encodes a protein that is involved in glycosylphosphatidylinositol (GPI)-anchor biosynthesis. The GPI-anchor is a glycolipid found on many blood cells and serves to anchor proteins to the cell surface. This protein is expressed in the endoplasmic reticulum and transfers phosphoethanolamine (EtNP) to the first mannose of the GPI anchor. Two alternatively spliced variants, which encode an identical isoform, have been reported. [provided by RefSeq, Jul 2008]
