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

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.\n\nApplications: \nSuitable for use in Western Blot and Immunohistochemistry. Other applications not tested.\n\nRecommended Dilution:\nImmunohistochemistry: 1:20-1:40 for 1 hr. at RT\nOptimal dilutions to be determined by the researcher.\n\nPositive Control: \nSkeletal muscle (for Immunohistochemistry: membrane staining of muscle fibers, also shows slight cytoplasmic labeling in a fiber-type mosaic)\n\nStorage and Stability:\nLyophilized 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.

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SPECIFICATIONS

Catalog Number

D9904-60F

Size

1ml

Applications

IHC, WB

Hosts

Mouse

Reactivities

Hum

Form

Supplied as a lyophilized powder in PBS, 1% BSA, 15mM sodium azide. Reconstitute with 1ml dH2O.

P Type

Mab

Purity

Supernatant

Isotype

IgG2b,k

References

1.Anderson LVB, Davison K, Moss J A, et al.. Dysferlin is a plasma membrane protein and is expressed early in human development. Human Molecular Genetics 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). Human Molecular Genetics 8:871-877 (1999).

Additional Info

Recognizes human Dysferlin.

Alternative Names

Dysferlin, Limb Girdle Muscular Dystrophy 2B (Autosomal Recessive)