| Product Name | Anti-LARGE1 Monoclonal Antibody |
|---|---|
| Description | LARGE1 serves as a glycosyltransferase which participates in glycosylation of the muscle membrane protein alpha-dystroglycan. Mutations of LARGE1 lead to hypoglycosylation of alpha-dystroglycan and cause congenital muscular dystrophy (MDC1D) associated with severe mental retardation. Altered alpha-dystroglycan glycosylation may also play a role in cancer, as hypoglycosylation of the protein and loss of laminin binding have been demonstrated in invasive carcinoma cells. The reagent is designed for Flow Cytometry analysis. |
| Clone | LARGE-02 |
| Immunogen | Recombinant fragment of human LARGE1 (amino acids 35-142) |
| Isotype | IgG2b |
| Specificity | The mouse monoclonal antibody LARGE-02 recognizes human LARGE1, a glycosyltransferase localizing mainly to the Golgi apparatus. Crossreactivity with LARGE2 was not determined. |
| Reactivity | Human |
| Applications | FC, WB |
| Form | The reagent is provided in stabilizing phosphate buffered saline (PBS) solution containing 15mM sodium azide. |
| Storage | Store in the dark at 2-8°C. Do not freeze. Avoid prolonged exposure to light. |
| Background | LARGE1 serves as a glycosyltransferase which participates in glycosylation of the muscle membrane protein alpha-dystroglycan. Mutations of LARGE1 lead to hypoglycosylation of alpha-dystroglycan and cause congenital muscular dystrophy (MDC1D) associated with severe mental retardation. Altered alpha-dystroglycan glycosylation may also play a role in cancer, as hypoglycosylation of the protein and loss of laminin binding have been demonstrated in invasive carcinoma cells. |
| Supplier | Exbio |
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