Anti-LARGE1 PE

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.

Product Specifications

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

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.