Anti-LARGE1 Purified

• 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 (QC tested), WB

• Concentration:

  1 mg/mL

• Format:

  Purified

• Buffer:

  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. 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