FKRP Rabbit Polyclonal Antibody

CAT#: TA354897

Rabbit anti FKRP Polyclonal Antibody

Product Images

WB: The whole mouse muscle tissue lysates were immunoblotted by Rabbit anti-FKRP at 1:500 (Lane 1). The immunoreactive band is blocked by pre-incubation of immunizing peptide (lane 2)

Specifications

Product Data

• Applications: WB
• Recommended Dilution: WB 0.1-1 µg/ml ELISA 0.01-0.1 µg/ml IP 2-5 µg/ml IHC 2-10 µg/ml FC 5-10 µg/ml
• Reactivities: Human, Mouse, Rat
• Host: Rabbit
• Isotype: IgG
• Clonality: Polyclonal
• Immunogen: A synthetic peptide corresponding to the C-terminus of human FKRP protein. This sequence is identical to human, mouse and rat.
• Formulation: This affinity purified antibody is supplied in sterile Phosphate buffered saline (pH7.2) containing antibody stabilizer.
• Purification: The Rabbit IgG is purified by Epitope Affinity Purification
• Conjugation: Unconjugated
• Storage: Store at -20°C as received.
• Stability: Stable for 12 months from date of receipt.
• Predicted Protein Size: 55 kDa
• Gene Name: fukutin related protein
• Database Link:
  NP_001034974
  Entrez Gene 243853 MouseEntrez Gene 308390 RatEntrez Gene 79147 Human
  UniProt ID Q9H9S5
• Background: 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.
• Synonyms: LGMD2I; MDC1C; MDDGA5; MDDGB5; MDDGC5

Reference Data

• Protein Families: Transmembrane