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Dysferlin (Dystrophy-associated Fer-1-like Protein, DYSF, Fer-1-like Protein 1, FER1L1, FLJ00175, FLJ90168, LGMD2B)

Dysferlin (Dystrophy-associated Fer-1-like Protein, DYSF, Fer-1-like Protein 1, FER1L1, FLJ00175, FLJ90168, LGMD2B)

Cat no: D9904-60D

Supplier: United States Biological
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Dysferlin, a 230kD skeletal muscle protein, is a novel member of Ferlin protein family. It consists of a transmembrane domain responsible for its localization in sarcolemma and C-terminal C2 domains responsible for calcium-dependent phospholipid binding and vesicle fusion. It binds to proteins like Caveolin-3 and affixin and its role has been implicated in muscle contraction, membrane regeneration and repair. Mutations in dysferlin result in Miyoshi myopathy, limb girdle muscular dystrophy 2B and distal anterior compartment myopathy. Northern Blot analysis detected highest expression of dysferlin in skeletal muscle, heart and kidney. Applications: Suitable for use in Immunohistochemistry. Other applications not tested. Recommended Dilution: Immunohistochemistry (paraffin): 1:50-1:100 Optimal dilutions to be determined by the researcher. Positive Control: Skeletal muscle Cellular Localization: Cytoplasmic and cell membrane Storage and Stability: May be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Catalogue number: D9904-60D
Reactivities: Human
Hosts: Rabbit
Applications: Immunohistochemistry
Size: 500ul
Form: Supplied as a liquid in 0.05% sodium azide.
P type: Pab
Isotype: IgG
Purity: Serum
Alternative names: Dysferlin, Limb Girdle Muscular Dystrophy 2B (Autosomal Recessive)
References: 1. Capanni et.al Exp Mol Med 35 (6) 538-544 (December 2003).
Additional info: Recognizes human Dysferlin.

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