Anti-LARGE1 Purified
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 (QC tested), WB
Concentration
1 mg/mL
Format
Purified
Buffer
Phosphate buffered saline (PBS), pH 7.4, 15 mM sodium azide
Other References
Storage Conditions
Store at 2-8°C. Do not freeze.
Specificity
Applications Notes
Flow cytometry: Recommended dilution: 1-5 μg/ml. Intracellular staining.
Isotype
Mouse IgG2b
Frequently Asked Questions
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