Species: | Rabbit |
Applications: | WB IHC IF |
Immunogen Range: | KLH conjugated synthetic peptide derived from human GMPPB |
Clonality: | Polyclonal Antibody |
Isotype: | IgG |
GENE ID: | 29925 |
Swiss Prot: | |
Synonyms: | GDP mannose pyrophosphorylase B, GDP-mannose pyrophosphorylase B, GMPPB, GMPPB_HUMAN, GTP-mannose-1-phosphate guanylyltransferase beta, KIAA1851, Mannose 1 phosphate guanylyltransferase, Mannose-1-phosphate guanyltransferase beta. |
Purification: | Purified by Protein A. |
Storage: | Aqueous buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% sodium azide. Store at -20℃ for 12 months |
Background: | GMPPB is a 360 amino acid protein that belongs to the transferase hexapeptide repeat family and is involved in protein modification pathways. Functioning as a GDP-mannose pyrophosphorylase, GMPPB enzymatically catalyzes the conversion of mannose-1-phosphate and GTP to GDP-mannose and a free phosphate, a reaction that is involved in the production of N-linked oligosaccharides. Defects in the gene encoding GMPPB that cause errors in the glycosylation pathway may lead to congenital disorders of glycosylation (CDG). CDGs are multisystemic diseases that often involve both the central and peripheral nervous systems and are often characterized by endocrine and coagulation disorders. GMPPB is expressed as two isoforms due to alternative splicing events. |
Caculated MW: | / |
Observed MW: | Refer to Figures |
Applications: |
WB 1:100-1:1000 IHC 1:100-1:500 IF 1:50-1:200 |
Reacitivity: | Human, Mouse, Rat |