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You are here:Home » Antibodies » Abs to Membrane Proteins » Anti -Dysferlin (Dystrophy-associated Fer-1-like Protein, DYSF, Fer-1-like Protein 1, FER1L1, FLJ00175, FLJ90168, LGMD2B)

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

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Specifications

Clone Host Grade Applications
Polyclonal Rabbit Serum IH
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.
Catalog #D9904-60D
ApplicationsSuitable for use in Immunohistochemistry. Other applications not tested.
Recommended DilutionImmunohistochemistry (paraffin): 1:50-1:100
Optimal dilutions to be determined by the researcher.
Positive ControlSkeletal muscle
Cellular Localization
Cytoplasmic and cell membrane
Storage and StabilityMay be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Clone TypePolyclonal
IsotypeIgG
HostRabbit
SourceHuman
ConcentrationNot determined
FormSupplied as a liquid in 0.05% sodium azide.
PuritySerum
ImmunogenSynthetic peptide corresponding to C-terminal of human Dysferlin.
SpecificityRecognizes human Dysferlin.
Important NoteThis product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications without the expressed written authorization of United States Biological.
Alternate namesDysferlin, Limb Girdle Muscular Dystrophy 2B (Autosomal Recessive)


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