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FKTN / Fukutin Antibody
Rabbit Polyclonal Antibody
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Application 
| WB, IHC-P, IF, ICC |
|---|---|
| Primary Accession | O75072 |
| Reactivity | Human |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 54kDa |
| Dilution | ICC (1:100-1:200), IF (1:100-1:200), IHC-P (10 µg/ml), WB (1:500-1:3000) , |
| Gene ID | 2218 |
|---|---|
| Other Names | Fukutin, 2.-.-.-, Fukuyama-type congenital muscular dystrophy protein, FKTN, FCMD |
| Target/Specificity | Human Fukutin. Predicted cross-reactivity based on amino acid sequence homology: mouse (90%), rat (91%). |
| Reconstitution & Storage | Aliquot and store at -20°C. Minimize freezing and thawing. |
| Precautions | FKTN / Fukutin Antibody is for research use only and not for use in diagnostic or therapeutic procedures. |
| Name | FKTN (HGNC:3622) |
|---|---|
| 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:27194101, PubMed:29477842). 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:27194101, PubMed:29477842). 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). |
| Cellular Location | Golgi apparatus membrane; Single-pass type II membrane protein. Cytoplasm {ECO:0000250|UniProtKB:Q8R507}. Nucleus {ECO:0000250|UniProtKB:Q8R507}. Note=In retinal tissue, does not localize with the Golgi apparatus. {ECO:0000250|UniProtKB:Q8R507} |
| Tissue Location | 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) |
| Volume | 50 µl |
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Provided below are standard protocols that you may find useful for product applications.
Background
Glycosyltransferase involved in the biosynthesis 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), which is required for binding laminin G-like domain-containing extracellular proteins with high affinity. May interact with and reinforce a large complex encompassing the outside and inside of muscle membranes. Could be involved in brain development.
References
Kobayashi K.,et al.Nature 394:388-392(1998).
Kobayashi K.,et al.FEBS Lett. 489:192-196(2001).
Ota T.,et al.Nat. Genet. 36:40-45(2004).
Humphray S.J.,et al.Nature 429:369-374(2004).
Saito K.,et al.Am. J. Med. Genet. 92:184-190(2000).
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