Dysferlin (DYSF) (C-term) Rabbit Polyclonal Antibody
Other products for "DYSF"
Specifications
Product Data | |
Applications | IHC, WB |
Recommended Dilution | Immunohistochemistry on Paraffin Sections: 1/50. Western Blot: 1 µg/ml. |
Reactivities | Human |
Host | Rabbit |
Isotype | IgG |
Clonality | Polyclonal |
Immunogen | A synthetic peptide corresponding to C-terminal of human Dysferlin |
Specificity | This antibody reacts to Dysferlin, Limb Girdle Muscular Dystrophy 2b (AUTOSOMAL RECESSIVE) (DYSF). |
Formulation | 10 mM PBS, pH 7.4 containing BSA as stabilizer and 0,09% sodium azide as preservative State: Purified State: Liquid purfied Ig fraction |
Purification | Immunoaffinity Chromatography |
Gene Name | Homo sapiens dysferlin (DYSF), transcript variant 2 |
Database Link | |
Background | Dysferlin is the protein product of the 2p13 gene that is defective in patients with Limb-Girdle Muscular Dystrophy type 2B (LGMD2B) and Miyoshi Myopathy (MM). Dysferlin is normally localized to the muscle plasma membrane. In patients with LGMD2B and MM, immunoreactivity to dysferlin is severely reduced or lost, depending on the type of mutation. This antibody is used for the characterization of LGMD2B and MM. |
Synonyms | Fer-1-like protein 1, DYSF, FER1L1 |
Reference Data | |
Protein Families | Transmembrane |
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