Fukutin-related protein Antibodies

AND FKRP ELISA kits, Fukutin-related protein Proteins

Many biological assays use antibodies to detect Fukutin-related protein, such as Western Blot (WB), ELISA, Flow Cytometry, Immunocytochemistry (IHC). These antibodies can be polyclonal or monoclonal, and react with Fukutin-related protein in Human, Mouse, Rat, and many other animal samples. Boster Bio use mainly mouse and rabbit to develop Fukutin-related protein antibodies...

We validate the specificity of these antibodies to Fukutin-related protein by testing them on tissues known to express FKRP positively and negatively. Browse below to find the FKRP antibody that suites your experiment. We have 2 of these antibodies and many publications and validation images.

If you cannot find antibodies that fit your needs, contact us for making custom antibodies. We have a full suite of custom antibody services covering from research to diagnostic and therapeutic applications.

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FKRP Infographic by Boster Bio

All FKRP Products

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6 Q&As

Human, Mouse, Rat

$360
Cat # A02889
100 µl

FKRP (NM_024301) Human Over-expression Lysate

Human

$628
Cat # LS079147
100 µg

FKRP Overview

Facts about Fukutin-related protein.

FKRP 3D structure. Source: alphafold

Fukutin-related protein (FKRP)

Catalyzes the transfer of CDP-ribitol to ribitol 5- phosphate formerly attached by FKTN/fukutin of into the phosphorylated O-mannosyl trisaccharide (N-acetylgalactosamine- beta-3-N-acetylglucosamine-beta-4-(phosphate-6-)mannose), a carbohydrate structure present in alpha-dystroglycan (DAG1) (PubMed:25279699, PubMed:26923585, PubMed:29477842). This constitutes the second step in the formation of the ribose 5- phosphate tandem repeat which links the phosphorylated O-mannosyl trisaccharide into the ligand binding moiety composed of repeats of 3-xylosyl-alpha-1,3-glucuronic acid-beta-1 (PubMed:25279699, PubMed:26923585, PubMed:29477842).

Gene Information

Human
Gene Name:	FKRP
Uniprot:	Q9H9S5
Entrez:	79147

Family

Belongs to:

LicD transferase family

Alternative Names

EC 2.-; fukutin related protein; fukutin-related protein; LGMD2IFLJ12576; MDC1CMGC2991; MDDGA5; MDDGB5; MDDGC5

Molecular Weight

Mass (kDA):
54.568 kDA

Genomic Context


Human

Location:	19q13.32
Sequence:	19; NC_000019.10 (46746015..46758575)

Tissue Specificity

Expressed in the retina (at protein level) (PubMed:29416295). Expressed predominantly in skeletal muscle, placenta, and heart and relatively weakly in brain, lung, liver, kidney, and pancreas (PubMed:11592034).

Subcellular Localizations

Golgi apparatus membrane; Single-pass type II membrane protein. Secreted. Cell membrane, sarcolemma. Rough endoplasmic reticulum. Cytoplasm. According to some studies the N-terminal hydrophobic domain is cleaved after translocation to the Golgi apparatus and the protein is secreted (PubMed:19900540). Localization at the cell membrane may require the presence of dystroglycan (By similarity). At the Golgi apparatus localizes to the middle-to-trans-cisternae, as assessed by MG160 colocalization. Detected in rough endoplasmic reticulum in myocytes (PubMed:17554798, PubMed:21886772). In general, mu

Diseases

Muscular Dystrophy
Dystrophy
Muscular Dystrophies, Limb-girdle
Congenital Muscular Dystrophy (disorder)
Walker-warburg Congenital Muscular Dystrophy
Muscle Eye Brain Disease
Coronary Microvascular Disease
Weakness
Myopathy
Muscle Weakness
Cardiomyopathies
Nervousness

Lissencephaly
Fukuyama Type Congenital Muscular Dystrophy
Hypoplasia
Hypertrophy
Eye Abnormalities
Muscular Dystrophy, Duchenne
Congenital Anomaly Of Brain

Pathways

Glycosylation
Pathogenesis
Localization
Secretion
Regeneration
Protein Glycosylation
Muscle Hypertrophy
Golgi Localization
Signal Transmission
Brain Development
Membrane Organization
Basement Membrane Organization

Protein Secretion
Inflammatory Response
Mrna Splicing
Angiogenesis
Rna Processing
Eye Development
Rna Interference

PTMs

Glycosylation

Phosphorylation

Cleavage

For details, visit:
bosterbio.com/bosterbio-gene-info-cards/FKRP

References

PMID: 11592034 by Brockington M., et al. Mutations in the fukutin-related protein gene (FKRP) cause a form of congenital muscular dystrophy with secondary laminin alpha2 deficiency and abnormal glycosylation of alpha-dystroglycan.

PMID: 15213246 by Matsumoto H., et al. Subcellular localization of fukutin and fukutin-related protein in muscle cells.