Anti-Fukutin-related protein FKRP Antibody

Boster Bio Anti-Fukutin-related protein FKRP Antibody (Catalog # A02889) . Tested in WB applications. This antibody reacts with Human, Mouse, Rat.

Product Specifications

Background

Could be a transferase involved in the modification of glycan moieties of alpha-dystroglycan (DAG1).<br><br>Brockington M., Am. J. Hum. Genet. 69:1198-1209(2001).<br>Ota T., Nat. Genet. 36:40-45(2004).<br>Brockington M., Hum. Mol. Genet. 10:2851-2859(2001).

Synonyms

Fukutin-related protein;2.-.-.-; FKRP

Gene Name

FKRP

UniProt

Q9H9S5

Host

Rabbit

Reactivity

Human, Mouse, Rat

Immunogen

Synthesized peptide derived from N-terminal of human FKRP.

Clonality

Polyclonal

Tissue Specificity

Expressed predominantly in skeletal muscle, placenta, and heart and relatively weakly in brain, lung, liver kidney and pancreas.

Applications

Field of Research

Golgi Proteins, Protein Trafficking, Signal Transduction

Purification

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration

1 mg/ml

Dilution

Western blotting: 1:500~1:3000

Form

Liquid

Function

Transferase 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. .

Molecular Weight

54568 MW

Shipping Conditions

Available

Storage Conditions

Store at -20°C for one year. For short term storage and frequent use, store at 4°C for up to one month. Avoid repeated freeze-thaw cycles.

Other Gene Names

Fukutin-related protein

Subcellular Location

Golgi apparatus membrane ; Single-pass type II membrane protein. Secreted. Cell membrane, sarcolemma . Rough endoplasmic reticulum. According to some studies the N- terminal hydrophobic domain is cleaved after translocation to the Golgi apparatus and the protein is secreted. Localization at the cell membrane may require the presence of dystroglycan. At the Golgi apparatus localizes to the middle-to-trans-cisternae, as assessed by MG160 colocalization. Detected in rough endoplasmic reticulum in myocytes. In general, mutants associated with severe clinical phenotypes are retained within the endoplasmic reticulum.