![AffiAB® Anti-LARGE1 Purified [LARGE-02]](/web/image/product.product/79476/image_1024/%5BAFG-EXB-0506%5D%20AffiAB%C2%AE%20Anti-LARGE1%20Purified%20%5BLARGE-02%5D?unique=06fe5f0)
Overview
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.
Specificity:
The mouse monoclonal antibody LARGE-02 recognizes human LARGE1, a glycosyltransferase expressed mainly in the Golgi apparatus. Crossreactivity with LARGE2 was not determined.
Antigen
LARGE1
Clone
LARGE-02
Species Reactivity
Human
Negative Species
N/A
Isotype
Mouse IgG2b
Immunogen
Recombinant fragment of human LARGE1 (amino acids 35-142)
Application
FC (QC tested), WB
Regulatory status
RUO
Concentration
1 mg/ml
Format
Purified
Storage / Stability
Store at 2-8°C. Do not freeze.
Storage Buffer
Phosphate buffered saline (PBS), pH 7.4, 15 mM sodium azide