FKRP Antibody (E-4): sc-374642

FKRP Antibody (E-4) is a mouse monoclonal IgG1 kappa light chain antibody that detects FKRP of mouse, rat, and human origin by western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), and enzyme-linked immunosorbent assay (ELISA). FKRP (E-4) antibody is available in both non-conjugated and various conjugated forms, including agarose, horseradish peroxidase (HRP), phycoerythrin (PE), fluorescein isothiocyanate (FITC), and multiple Alexa Fluor® conjugates. Fukutin-related protein (FKRP) is a crucial glycosyltransferase predominantly expressed in heart, skeletal muscle, and placenta, with lower levels found in lung, liver, brain, kidney, and pancreas. FKRP localization to the medial Golgi apparatus is essential for glycosylating α-dystroglycan, a process vital for maintaining muscle integrity and function. Disruptions in FKRP localization due to mutations can lead to severe consequences, including various forms of congenital muscular dystrophy and related disorders, highlighting FKRP′s importance in both muscle and neurological health. FKRP′s role in glycosylation extends to involvement in diseases such as limb-girdle muscular dystrophy type 2I and muscle-eye-brain disease, where FKRP mislocalization from the Golgi apparatus can exacerbate these conditions.

Alexa Fluor^® is a trademark of Molecular Probes Inc., OR., USA

LI-COR^® and Odyssey^® are registered trademarks of LI-COR Biosciences

FKRP Antibody (E-4) References:

1. 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.  |  Brockington, M., et al. 2001. Am J Hum Genet. 69: 1198-209. PMID: 11592034
2. Fukutin-related protein mutations that cause congenital muscular dystrophy result in ER-retention of the mutant protein in cultured cells.  |  Esapa, CT., et al. 2005. Hum Mol Genet. 14: 295-305. PMID: 15574464
3. Dilated cardiomyopathy may be an early sign of the C826A Fukutin-related protein mutation.  |  Müller, T., et al. 2005. Neuromuscul Disord. 15: 372-6. PMID: 15833432
4. Mutated fukutin-related protein (FKRP) localises as wild type in differentiated muscle cells.  |  Dolatshad, NF., et al. 2005. Exp Cell Res. 309: 370-8. PMID: 16055117
5. Clinical and molecular characterization of patients with limb-girdle muscular dystrophy type 2I.  |  Boito, CA., et al. 2005. Arch Neurol. 62: 1894-9. PMID: 16344347
6. Walker-Warburg syndrome.  |  Vajsar, J. and Schachter, H. 2006. Orphanet J Rare Dis. 1: 29. PMID: 16887026

Monoclonal antibodies for mammalian and non-mammalian protein targets represent essentially all targets covered by polyclonal antibodies. Over 8,300 of our antibodies are offered as ImmunoCruz^® conjugates which eliminates the need for a secondary antibody.

Santa Cruz Biotechnology antibodies have over 360,000 research citations. Anti-FKRP Antibody (E-4) has 1 citations in a variety of scientific publications.

Primary antibodies like Anti-FKRP Antibody (E-4) for mammalian target proteins are recommended for the detection of a range of mammalian species, primarily of mouse, rat and human species. Many mammalian antibodies are also ideal for veterinary research and reactive with ovine, porcine, caprine, feline, canine, bovine and equine protein targets. Santa Cruz Biotechnology primary monoclonal antibodies are suitable for laboratory research applications and are not intended for diagnostic or therapeutic use.

FKRP Antibody (E-4) Specifications

Alternate Product Name: FKRP

Product Clone Name: E-4

Classification: Primary Antibody

Clonality: Monoclonal Antibody

Antibody Isotype: IgG₁ kappa light chain

Amino Acids: 368-495

Epitope Target: FKRP

Epitope Species: human

Product Reactivity/Detection: FKRP

Species Reactivity/Detection: mouse, rat, human

Additional Mammalian Reactivity: N/A

Additional Non-Mammalian Reactivity: N/A

Applications: WB, IP, IF, ELISA

Vialing Information: Each vial contains 200 µg IgG₁ kappa light chain in 1.0 ml of PBS with < 0.1% sodium azide and 0.1% gelatin

Research Citations: 1 citations in various scientific publications

Available Conjugates/Forms for FKRP Antibody (E-4): AC: Agarose, AF488: Alexa Fluor^® 488, AF546: Alexa Fluor^® 546, AF594: Alexa Fluor^® 594, AF647: Alexa Fluor^® 647, AF680: Alexa Fluor^® 680, AF790: Alexa Fluor^® 790, FITC: Fluoroscein, HRP: Horseradish Peroxidase, PE: Phycoerythrin