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Home / Antibodies

FKRP Antibody

SKU : BHA10558030

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CUSABIO TECHONOLOGY LLC

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Mfr.No.	CSB-PA035204
Alternative Names	Fukutin-related protein; EC 2.-.-.-; FKRP;
Categories	Primary Antibodies
Clonality	polyclonal
Description	Could be a transferase involved in the modification of glycan moieties of alpha-dystroglycan (DAG1).Brockington M., Am. J. Hum. Genet. 69:1198-1209(2001).Ota T., Nat. Genet. 36:40-45(2004).Brockington M., Hum. Mol. Genet. 10:2851-2859(2001).
Host	Rabbit
Immunogen	Synthesized peptide derived from N-terminal of Human FKRP.
Involvement In Disease	Muscular dystrophy-dystroglycanopathy congenital with brain and eye anomalies A5 (MDDGA5); Muscular dystrophy-dystroglycanopathy congenital with or without mental retardation B5 (MDDGB5); Muscular dystrophy-dystroglycanopathy limb-girdle C5 (MDDGC5)
Raised In	Rabbit
Reactivity	Human, Mouse, Rat
Regulatory	RUO
Relevance	Could be a transferase involved in the modification of glycan moieties of alpha-dystroglycan (DAG1). Brockington M., Am. J. Hum. Genet. 69:1198-1209(2001). Ota T., Nat. Genet. 36:40-45(2004). Brockington M., Hum. Mol. Genet. 10:2851-2859(2001).
Species	Homo Sapiens (Human)
Specificity	The antibody detects endogenous levels of total FKRP protein.
Subcellular Location	Golgi apparatus membrane, Single-pass type II membrane protein, Secreted, Cell membrane, sarcolemma, Rough endoplasmic reticulum

Uniprot	Q9H9S5
Database Links	HGNC:17997 OMIM:606596 KEGG:hsa:79147 STRING:9606.ENSP00000326570 UniGene:Hs.515493
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.
Protein Families	LicD transferase family
Tissue Specificity	Expressed predominantly in skeletal muscle, placenta, and heart and relatively weakly in brain, lung, liver kidney and pancreas.

Buffer	Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Form	Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Format	liquid
Purification	The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Purity	The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Storage	Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Applications

ELISA, WB

Description

ELISA ; WB, WB ; ELISA

Dilution

WB	1:500-1:3000

Tested Application

ELISA,WB;WB:1:500-1:3000

Datasheet
ELISA Protocol
Western Blotting(WB) Protocol

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SKU	Supplier No.	Size	Your price	Quantity
BHA10558030	CSB-PA035204	100ul	$297		Add to Cart

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