This gene encodes a protein which is targeted to the medial Golgi apparatus and is necessary for posttranslational modification of dystroglycan.
Mutations in this gene have been associated with congenital muscular dystrophy, mental retardation, and cerebellar cysts.
Several alternatively spliced transcript variants of this gene have been described, but the full-length nature of some of these variants has not been determined.
[provided by RefSeq].
Applications:Suitable for use in Western Blot and ELISA.
Other applications not tested.
Recommended Dilutions:Western Blot: 1:1000Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size:200ul
Host:rabbit
Source Antibody:human
Grade:Affinity Purified
Purity:Purified by Protein A and peptide affinity chromatography
Form:Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide
Specificity:Rcognizes human FKRP
Isotype:IgG
Calc Applications Abbrev:E WB
Calc Crossreactivity:Hu
Immunogen:KLH-conjugated synthetic peptide mapping to a fragment of residues within amino acids 237-265 in the central region of human FKRP