FKRP Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	WB
Recommend 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
Reactivity	Human, Mouse, Rat
Host	Rabbit
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 Condition	Store at -20°C as received.
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

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