Dysferlin (Romeo) (DYSF) antibody 
RabMAb®
Cat.#: 5140-1
Rabbit Monoclonal Antibody
Swiss Prot: O75923
Mol Weight: 280kDa
Price: $260*
Availability: Ship next business day
Description
Dysferlin is a 230 kDa single-pass type II transmembrane protein; it is a homolog to C. elegans FER1 protein, which mediates fusion of intracellular vesicles to the sperm plasma membrane (1). Dysferlin is highly expressed in skeletal muscle and commonly localized to the muscle plasma membrane. A mutation that causes muscular dystrophy weakens calcium dependent binding of the C2 domain of Dysferlin to phospholipid (2). No or low immunoreactivity to Dysferlin is seen in Limb-Girdle Muscular Dystrophy type 2B (LGMD2B) and Miyoshi Myopathy (MM).
Recommended Applications
WB, IHC, ICC
Applications and Recommended Dilution Factors
WB: 1:1,000 - 10,000
IHC: 1:50 - 100
ICC: 1:200 - 400
Species Reactivity *
Human, Mouse
*Species cross-reactivity is based on WB analysis.
Products Data
 |  |  |
| Western blot analysis of human control, human LGMD 2B, mouse wild type, and mouse Dysf-/- skeletal muscle homogenates using anti-Dysferlin RabMAb (cat. #5140-1). The wild type and Dysf-/- mice were purchased from The Jackson Laboratory. The control strain is 129S1/SvImJ (stock number 002448). The Dysf-/- strain is 129-Dysftm1Kcam/J. The donating investigator for the Dysf-/- strain (Nature 423:168-172, 2003) is Kevin P. Campbell, The University of Iowa. Images are kindly provided by Dr. Steven A. Moore from University of Iowa. | Immunohistochemical analysis of paraffin-embedded human muscle tissue using anti-Dysferlin RabMAb (cat. #5140-1). | Immunofluorescence image of cat. #5140-1 on unfixed frozen sections from normal control human skeletal muscle.
Images are kindly provided by Dr. Steven A. Moore from University of Iowa. |
 |  |  |
| Immunofluorescence image of cat. #5140-1 on unfixed frozen sections of skeletal muscle from a patient with LGMD 2B.
Images are kindly provided by Dr. Steven A. Moore from University of Iowa. | Immunofluorescence image of cat. #5140-1 on unfixed frozen sections from wild type mouse skeletal muscle. This control mouse strain is 129S1/SvImJ (stock number 002448) from The Jackson Laboratory.
Images are kindly provided by Dr. Steven A. Moore from University of Iowa. | Immunofluorescence image of cat. #5140-1 on unfixed frozen sections from Dysf-/- mouse skeletal muscle. This Dysf-/- strain is 129-Dysftm1Kcam/J from The Jackson Laboratory. The donating investigator for the Dysf-/- strain (Nature 423:168-172, 2003) is Kevin P. Campbell, The University of Iowa.
Images are kindly provided by Dr. Steven A. Moore from University of Iowa. |
Specificity
A synthetic peptide corresponding to residues in human Dysferlin was used as an immunogen.
This antibody was made in collaboration with the Jain Foundation whose goal is to hasten EVERY avenue that may lead to the cure for LGMD2B/Miyoshi.
Storage Condition and Buffer
Store at -20 °C. Buffer: Antibody buffer, sodium azide, glycerol, and BSA. Stable for 12 months from date of receipt.
Alternative Names
DYSF antibody, FER1L1 antibody, FLJ00175 antibody, FLJ90168 antibody, LGMD2B antibody, Dysferlin antibody, Dystrophy-associated fer-1-like protein antibody, Fer-1-like protein 1 antibody
Description References
1. Achanzar WE, Ward S, J. Cell Sci. 110:1073–1081, 1997
2. Davis DB et al., J. Biol. Chem. 277:22883–22888, 2002
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