Application
Western blot analysis of POMGnT1 expression in NIH/3T3 whole cell lysate.| Product Name | POMGnT1 Antibody (H-300) |
|---|---|
| Antibody Type | Primary Antibodies |
| Antigen Alias | epitope corresponding to amino acids 1-300 mapping at the N-terminus of POMGnT1 of human origin |
| Modification Notes | The product of the POMGNT1 gene, protein O-mannose beta-1,2-N-acetylglucosaminyltransferase, participates in O-mannosyl glycan synthesis. POMGnT1 is an N(in)/C(out) (type II) membrane protein localized in the medial-Golgi that initiates the conversion of high mannose N-glycans to complex N-glycans. Specifically, POMGnT1 is a glycosylation enzyme that participates in the synthesis of O-mannosyl glycan, a laminin-binding ligand of alpha-dystroglycan that is rarely synthesized in mammals. Mutations in the POMGNT1 gene cause muscle-eye-brain disease (MEB), an autosomal recessive disorder characterized by congenital muscular dystrophy, ocular abnormalities and lissencephaly. Altered glycosylation of alpha-dystroglycan may play a critical role in the pathomechanism of MEB as well as Walker-Warburg syndrome (WWS), characterized by the absence of glycosylation of alpha-dystroglycan. The human POMGNT1 gene maps to chromosome 1p34-p33 and encodes a 660-amino acid type II transmembrane protein. |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | WBIPIFELISA |
| recommended for detection of POMGnT1 of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including canine, bovine and porcine: |
|
| Species Reactivity | HumanMouseRat |
| Concentration | 1mg/ml |
| Purification | Affinity purified |
| Alternative Names | epitope corresponding to amino acids 1-300 mapping at the N-terminus of POMGnT1 of human origin |
|---|---|
| Tissue Specificity | epitope corresponding to amino acids 1-300 mapping at the N-terminus of POMGnT1 of human origin |
| Entrez Gene | 55624 |
|---|
Application
Western blot analysis of POMGnT1 expression in NIH/3T3 whole cell lysate.| Application Notes | recommended for detection of POMGnT1 of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including canine, bovine and porcine: |
|---|
| Form | Liquid |
|---|---|
| Storage Instructions | For short-term storage, store at 4° C. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles. |
| Storage Buffer | phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. |
