Antigen 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. Patients with other neuromuscular conditions demonstrate normal labeling patterns. Product Specific Information HAMLE...

Antigen Background Duchenne Muscular dystrophy (DMD) is the most common of the muscular dystrophies resulting in progressive muscular wasting and death. Dystrophin is the 427kD protein product of the DMD gene located on the X chromosome at position Xp21. Abnormalities in protein expression occur in patients with DMD/BMD and dystrophin analysis may be used to distinguish these conditions from other neuromuscular diseases. Severe Duchenne muscular ...

Antigen Background Mucins are heavily glycosylated glycoproteins which constitute the major components of mucus that covers the surface of epithelial tissues. Nine distinct epithelial mucin genes (Muc-1, 2, 3, 4, 5AC, 5B, 6, 7 and 8) have been identified. Various immunohistochemical and in situ hybridization studies have shown that these mucins are differentially expressed in epithelia with cell-type specificity. The normal gastric mucosa shows c...

Antigen Background Myosin is a contractile muscle specific protein composed of two heavy and four light chains. The myosin heavy chain has many isoforms which are specific for different muscles or fiber types, some of which are developmentally regulated. The range of myosin heavy chain antibodies may prove useful for investigating development of intrafusal and extrafusal muscle fibers and the course of muscle fiber regeneration. At the ultrastruc...

Antigen Background The gene responsible for LGMD2A has been identified as the chromosome 15q15-encoded muscle-specific calcium-activated neutral protease, calpain 3. Calpain 3 enzyme is only stable in human muscle when homogenized in treatment buffer immediately after harvest (Anderson LVB et al. Am. J. of Pathol. 153(4), 1169-1179 (1998)), and in homogenates containing SDS and is therefore well suited for analysis by Western blot. NCL-CALP-2C4 r...

Antigen Background In normal skeletal muscle, dystrophin, the protein product of the gene which is defective in Duchenne and Becker muscular dystrophy, is attached to the muscle membrane via a complex of proteins (dystrophin-associated glycoproteins, DAGs). Dystrophin-deficient muscle shows a generalized reduction in DAG labeling. The expression of different members of the dystrophin glycoprotein complex is altered in several types of muscular dy...

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