Anti-FKRP Polyclonal Antibody
Product Sizes
100ug
£597.00
OAAI00760-100UG
About this Product
- SKU:
- OAAI00760
- Additional Names:
- FKTR;fukutin-related protein;LGMD2I;LGMDR9;MDC1C;MDDGA5;MDDGB5;MDDGC5;ribitol-5-phosphate transferase.
- Clonality:
- Polyclonal
- Concentration:
- 100ug/200ul
- Extra Details:
- Fukutin-related protein (FKRP) 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. Mutations in fukutin-related protein (FKRP) give rise to mild and more severe forms of muscular dystrophy.
FKRP may influence the glycosylation status of dystroglycan from within the sarcolemmal dystrophin-glycoprotein complex.
FKRP associates with the sarcolemmal DGC and that it plays a unique role in dystroglycanopathy muscle disease. - Gene Details:
- fukutin related protein
- Host:
- Rabbit
- Immunogen:
- A synthetic peptide corresponding to the C-terminus of human FKRP protein. This sequence is identical to human; mouse and rat
- Isotype:
- Rabbit IgG
- Purification:
- Affinity Purified
- Shipping Conditions:
- Blue Ice
- Storage Conditions:
- The antibodies are stable for 24 months from date of receipt when stored at -20C to -70C. The antibodies can be stored at 2C-8C for three month without detectable loss of activity. Avoid repeated freezing-thawing cycles.
- Supplier:
- Aviva Systems Biology
- Type:
- Antibodies: Polyclonal Antibody
- Manufacturer's Data Sheet:html_datasheet.php
