Anti-LARGE1 PE

• 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.

• Certification :

  RUO

• Reactivity :

  Human

• Immunogen :

  Recombinant fragment of human LARGE1 (amino acids 35-142)

• Target Antigen :

  LARGE1

• Clone :

  LARGE-02

• Applications :

  FC-IC (QC tested)

• Concentration :

  0.1 mg/mL

• Format :

  PE

• Buffer :

  Stabilizing phosphate buffered saline (PBS), pH 7.4, 15 mM sodium azide

• Other References :

  *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., URL: http://www.ncbi.nlm.nih.gov/pubmed/15958417

• Storage Conditions :

  Store at 2-8°C. Protect from prolonged exposure to light. Do not freeze.

• Specificity :

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

• Applications Notes :

  Flow cytometry: Recommended dilution: 1-5 μg/ml. Intracellular staining.

• Isotype :

  Mouse IgG2b