dysferlin Antibody (C-11): sc-398905

dysferlin Antibody (C-11) is a mouse monoclonal IgG3 antibody that detects dysferlin protein of human origin by western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), and enzyme-linked immunosorbent assay (ELISA). anti-dysferlin antibody (C-11) is available as a non-conjugated format. Dysferlin plays a crucial role in muscle function and development, as dysferlin is essential for the repair of muscle fibers following injury. Dysferlin is located at the muscle fiber membrane, where dysferlin facilitates the fusion of vesicles containing membrane components to the plasma membrane, a process vital for maintaining muscle integrity and function. Mutations in the human dysferlin gene, DYSF, which is situated on chromosome 2p13.3-p13.1, are linked to limb girdle muscular dystrophy-2B (LGMD-2B) and Miyoshi myopathy (MM), both of which lead to progressive muscle weakness. Dysferlin is first detected in 5-6 week embryos, coinciding with the onset of limb formation, and while dysferlin is not essential for the initial stages of muscle development, dysferlin′s presence is critical for the sustained function of mature muscle. Absence of dysferlin during development can lead to the manifestation of disease phenotypes in adulthood, highlighting dysferlin′s importance in muscle health. Identical mutations in the DYSF gene can result in various myopathy phenotypes, suggesting that other genetic factors may also influence the clinical outcomes. The DYSF gene is unique, lacking homology to any other known mammalian gene, yet dysferlin shares similarities with the spermatogenesis factor fer-1 found in Caenorhabditis elegans, which is reflected in the name ′dysferlin′ that signifies dysferlin′s association with muscular dystrophy.

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dysferlin Antibody (C-11) References:

1. Dysferlin is a plasma membrane protein and is expressed early in human development.  |  Anderson, LV., et al. 1999. Hum Mol Genet. 8: 855-61. PMID: 10196375
2. Identical mutation in patients with limb girdle muscular dystrophy type 2B or Miyoshi myopathy suggests a role for modifier gene(s).  |  Weiler, T., et al. 1999. Hum Mol Genet. 8: 871-7. PMID: 10196377
3. Dysferlin is a surface membrane-associated protein that is absent in Miyoshi myopathy.  |  Matsuda, C., et al. 1999. Neurology. 53: 1119-22. PMID: 10496277
4. A gene for autosomal recessive limb-girdle muscular dystrophy maps to chromosome 2p.  |  Bashir, R., et al. 1994. Hum Mol Genet. 3: 455-7. PMID: 8012357
5. Dysferlin, a novel skeletal muscle gene, is mutated in Miyoshi myopathy and limb girdle muscular dystrophy.  |  Liu, J., et al. 1998. Nat Genet. 20: 31-6. PMID: 9731526

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-dysferlin Antibody (C-11) has 0 citations in a variety of scientific publications.

Primary antibodies like Anti-dysferlin Antibody (C-11) 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.

dysferlin Antibody (C-11) Specifications

Alternate Product Name: dysferlin

Product Clone Name: C-11

Classification: Primary Antibody

Clonality: Monoclonal Antibody

Antibody Isotype: IgG₃

Amino Acids: 84-150

Epitope Target: dysferlin

Epitope Species: human

Product Reactivity/Detection: dysferlin

Species Reactivity/Detection: human

Additional Mammalian Reactivity: N/A

Additional Non-Mammalian Reactivity: N/A

Applications: WB, IP, IF, ELISA

Vialing Information: Each vial contains 200 µg IgG₃ in 1.0 ml of PBS with < 0.1% sodium azide and 0.1% gelatin

Research Citations: 0 citations in various scientific publications

Available Conjugates/Forms for dysferlin Antibody (C-11): N/A