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Recombinant Mouse Fukutin-related protein (Fkrp), partial

Recombinant Mouse Fukutin-related protein (Fkrp), partial

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MSDS	MSDS Datasheet
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Source
Conjugate
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Target Background

Product Details

Abbreviation

Fkrp

Purity

>85% (SDS-PAGE)

Target Names

Fkrp

Uniprot NO.

Q8CG64

Alternative Names

FkrpFukutin-related protein; EC 2.4.2.-; Ribitol-5-phosphate transferase

Species

Mus musculus (Mouse)

Source

Yeast

Protein Length

Partial

Tag Info

N-terminal His-tagged/Tag-Free
The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.

Storage Condition

The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.

Shelf Life

The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.

Notes

Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.

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Target Background

Function(From Uniprot)

Catalyzes the transfer of CDP-ribitol to ribitol 5-phosphate previously attached by FKTN/fukutin of to the phosphorylated O-mannosyl trisaccharide (N-acetylgalactosamine-beta-3-N-acetylglucosamine-beta-4-(phosphate-6-)mannose), a carbohydrate structure present in alpha-dystroglycan (DAG1). This constitutes the second step in the formation of the ribose 5-phosphate tandem repeat which links the phosphorylated O-mannosyl trisaccharide to the ligand binding moiety composed of repeats of 3-xylosyl-alpha-1,3-glucuronic acid-beta-1.

Gene References into Functions

This study demonstrated that the Expression of glycosylated alpha-dystroglycan in newborn skeletal and cardiac muscles with FKRP mutation mice. PMID:27515093
Reduced Fkrp levels in skeletal muscle are associated with a progressive muscular dystrophy PMID:24234655
FKRP mutations are associated with muscular dystrophy. PMID:23591631
a reduction in Fkrp influences the ability of tissue-specific forms of alpha-dystroglycan to direct the deposition of several laminin isoforms PMID:21900571
investigation of the functional roles of FKRP in muscular dystrophies; FKRP is essential for the functional glycosylation of alpha-dystroglycan;both the mutation itself and the levels of FKRP expression are equally critical for the survival of the animals PMID:20675713
Basement membrane (BM) fragility may underlie abnormal phenotypes in fukutin-null embryos, and maintenance of BM function may require fukutin-mediated glycosylation of alpha-dystroglycan early in embryonic development. PMID:15837576
data offer the first evidence of a fukutin-related protein (FKRP) complex in muscle and suggest that FKRP may influence the glycosylation status of dystroglycan from within the sarcolemmal dystrophin-glycoprotein complex PMID:17452335
results suggest the generation of a mouse model for FKRP related muscular dystrophy requires a knock-down rather than a knock-in strategy in order to give rise to a disease phenotype. PMID:19155270

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Subcellular Location

Golgi apparatus membrane; Single-pass type II membrane protein. Secreted. Cell membrane, sarcolemma. Rough endoplasmic reticulum. Cytoplasm.

Protein Families

LicD transferase family

Tissue Specificity

Expressed in the retina, specifically in the inner segments of the photoreceptors, the outer plexiform layers, inner nuclear layers, and ganglion cell layers (at protein level). Expressed at highest levels in brain, lung, heart, kidney and liver.

Database Links

UNIGENE: Mm.39703

KEGG: mmu:243853

STRING: 10090.ENSMUSP00000059091

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