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Anti-LARGE1 PE

CAT: 0270-1P-702-C100Size: 0.1 mgDry Ice: NoHazardous: No
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CAT#:0270-1P-702-C100Size:0.1 mg
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24/48H Stock Items & 2 to 6 Weeks non Stock Items.
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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

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