Mouse Monoclonal to LARGE1
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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 mouse monoclonal antibody LARGE-02 recognizes human LARGE1, a glycosyltransferase localizing mainly to the Golgi apparatus. Crossreactivity with LARGE2 was not determined.
Recombinant fragment of human LARGE1 (amino acids 35-142)
- Flow Cytometry
- Western Blotting
Indicated dilutions are recommended starting points for use of this product. Working concentrations should be determined by the investigator.
*Grewal PK, McLaughlan JM, Moore CJ, Browning CA, Hewitt JE: Characterization of the LARGE family of putative glycosyltransferases associated with dystroglycanopathies. Glycobiology. 2005 Oct;15(10):912-23. [Abstract] [Full Text]
For research use only. Not for drug, diagnostic or other use.
Western blotting analysis of LARGE1 in HEK293-LARGE1 transfectants (A) and HEK293 cells (B) using mouse monoclonal antibody (clone LARGE-02
Flow cytometry analysis of LARGE1 in HEK293-LARGE1 transfectants using mouse monoclonal antibody (clone LARGE-02