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Western blot(SDS PAGE) analysis of extracts from HeLa cell lysate.Using Fukutin Rabbit mAb [3078]at dilution of 1:1000 incubated at 4℃ over night.
Protein names :CMD1X; FCMD; FKTN; Fukutin; LGMD2M; MDDGA4; MDDGB4; MDDGC4;
UniProtID :O75072
MASS(da) :53,724
MW(kDa) :51kDa
Form :Liquid
Purification :Affinity-chromatography
Host :Rabbit
Isotype : IgG
sensitivity :Endogenous
Reactivity :Human,Mouse,Rat
Specificity :Antibody is produced by immunizing animals with A synthesized peptide derived from human Fukutin
Storage :Antibody store in 10 mM PBS, 0.5mg/ml BSA, 50% glycerol. Shipped at 4°C. Store at-20°C or -80°C. Products are valid for one natural year of receipt.Avoid repeated freeze / thaw cycles.
WB Positive detected :HeLa cell lysate.
Function : Catalyzes the transfer of a ribitol-phosphate from CDP-ribitol to the distal N-acetylgalactosamine of the phosphorylated O-mannosyl trisaccharide (N-acetylgalactosamine-beta-3-N-acetylglucosamine-beta-4-(phosphate-6-)mannose), a carbohydrate structure present in alpha-dystroglycan (DAG1) (PubMed:26923585, PubMed:29477842, PubMed:27194101). This constitutes the first step in the formation of the ribitol 5-phosphate tandem repeat which links the phosphorylated O-mannosyl trisaccharide to the ligand binding moiety composed of repeats of 3-xylosyl-alpha-1,3-glucuronic acid-beta-1 (PubMed:17034757, PubMed:25279699, PubMed:26923585, PubMed:29477842, PubMed:27194101). Required for normal location of POMGNT1 in Golgi membranes, and for normal POMGNT1 activity (PubMed:17034757). May interact with and reinforce a large complex encompassing the outside and inside of muscle membranes (PubMed:25279699). Could be involved in brain development (Probable)..
Tissue specificity :Expressed in the retina (at protein level) (PubMed:29416295). Widely expressed with highest expression in brain, heart, pancreas and skeletal muscle (PubMed:11115853). Expressed at similar levels in control fetal and adult brain (PubMed:11115853). Expressed in migrating neurons, including Cajar-Retzius cells and adult cortical neurons, as well as hippocampal pyramidal cells and cerebellar Purkinje cells (PubMed:11115853). No expression observed in the glia limitans, the subpial astrocytes (which contribute to basement membrane formation) or other glial cells (PubMed:11115853)..
Subcellular locationi :Golgi apparatus membrane,Single-pass type II membrane protein. Cytoplasm. Nucleus.
IMPORTANT: For western blots, incubate membrane with diluted primary antibody in 1% w/v BSA, 1X TBST at 4°C overnight.