特異性

The mouse monoclonal antibody LARGE-02 recognizes human LARGE1 antigen, a glycosyltransferase localizing mainly to the Golgi apparatus. Crossreactivity with LARGE2 was not determined.

抗原情報

LARGE1 serves as a glycosyltransferase which participates in glycosylation of the muscle membrane protein α-dystroglycan. Mutations of LARGE1 lead to hypoglycosylation of α-dystroglycan and cause congenital muscular dystrophy (MDC1D) associated with severe mental retardation. Altered α-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.

利用方法

保存方法

Avoid prolonged exposure to light. Store in the dark at 2-8°C. Do not freeze.

安定性情報

Do not use after expiration date stamped on vial label.

レファレンス

• 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. <·PMID:·15958417·>