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Dysferlin (Dystrophy-associated Fer-1-like Protein, DYSF, Fer-1-like Protein 1, FER1L1, LGMD2B, MMD1)

Dysferlin (Dystrophy-associated Fer-1-like Protein, DYSF, Fer-1-like Protein 1, FER1L1, LGMD2B, MMD1)

Cat no: D9904-60E

Supplier: United States Biological
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Dysferlin is found in normal muscle but has a differential expression in certain instances of muscle dystrophy. This antibody will help detect the presence or absence of dysferlin. Applications: Suitable for use in Immunohistochemistry. Other applications not tested. Recommended Dilution: Immunohistochemistry: 1:20-1:40 for 1 hr. at RT Optimal dilutions to be determined by the researcher. Positive Control: Normal human skeletal muscle. Membrane staining of muscle fibers. Also some cytoplasmic labeling in a fiber-type mosaic. Storage and Stability: Lyophilized powder may be stored at -20 degrees C. Stable for 12 months at -20 degrees C. Reconstitute with sterile ddH2O. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Reconstituted product is stable for 12 months at -20 degrees C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.
Catalogue number: D9904-60E
Reactivities: Human, Rat, Canine, Porcine, Rabbit
Hosts: Mouse
Applications: Immunohistochemistry
Size: 1ml
Form: Supplied as a lyophilized powder in PBS, 1% BSA, 15mM sodium azide. Reconstitute with 1ml dH2O.
P type: Mab
Isotype: IgG1
Purity: Supernatant
Alternative names: Dysferlin, Limb Girdle Muscular Dystrophy 2B (Autosomal Recessive)
References: 1.Anderson L V B, Davison K, Moss J A, et al.. Dysferlin is a plasma membrane protein and is expressed early in human development. Hum. Mol. Genet. 8:855-861 (1999). 2.Anderson L V B and Davison K. Multiplex western blotting system for the analysis of muscular dystrophy proteins. American Journal of Pathology . 154(4):1017-1022 (1999). 3.Bittner R E, Anderson L V B, Burkhardt E, et al.. Dysferlin deletion in SJL mice (SJL-Dysf) defines a natural model for limb girdle muscular dystrophy 2B. Nature Genetics. 23:141-142 (1999). 4.Weiler T, Bashir R, Anderson L V B et al.. Identical mutations in patients with limb girdle muscular dystrophy type 2B or miyoshi myopathy suggests a role for modifier gene(s). Hum. Mol. Genet. 8:871-877 (1999).
Additional info: Recognizes human Dysferlin. Species Crossreactivity: Canine, hamster, porcine, rabbit and rat.

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