Desmin (clone DES-82), anti-human, mouse, rat, pig
€378.00
In stock
SKU
251743
Catalog Nr.: 251743
Size: 0.1 mg
Isotype: Mouse IgG1
Applications: E, WB, IHC
Size: 0.1 mg
Isotype: Mouse IgG1
Applications: E, WB, IHC
Protein Family: Cytoskeleton Proteins
Pathway and Disease: Neurodegenerative Disorders
Description:
Desmin is a class-III intermediate filament found in muscle cells. In adult striated muscle desmin forms a fibrous network connecting myofibrils to each other and to the plasma membrane from the periphery of the Z-line structures. Defects in desmin are the cause of myopathy myofibrillar desmin-related (MFM-DES), also known as desmin-related myopathy (DRM). DRM is a neuromuscular disorder characterized by skeletal muscle weakness associated with cardiac conduction blocks, arrhythmias, restrictive heart failure, and by myofibrillar destruction with intracytoplasmic accumulation of desmin-reactive deposits in cardiac and skeletal muscle cells.
Alternate Names: DES, Desmin
Application Notes: E: 1:500-1:1,000; WB: 1:100-1:500; IHC: 1:100-1:500
Accession No.: P17661
Antigen: Desmin from pig stomach.
Format: Each vial contains 0.1 mg IgG in 0.1 ml (1 mg/ml) of PBS pH7.4, 2% BSA with 0.09% sodium azide. Antibody was purified by Protein-G affinity chromatography.
Storage:
Store at -20°C. Minimize freeze-thaw cycles. Product is guaranteed one year from the date of shipment.
Pathway and Disease: Neurodegenerative Disorders
Description:
Desmin is a class-III intermediate filament found in muscle cells. In adult striated muscle desmin forms a fibrous network connecting myofibrils to each other and to the plasma membrane from the periphery of the Z-line structures. Defects in desmin are the cause of myopathy myofibrillar desmin-related (MFM-DES), also known as desmin-related myopathy (DRM). DRM is a neuromuscular disorder characterized by skeletal muscle weakness associated with cardiac conduction blocks, arrhythmias, restrictive heart failure, and by myofibrillar destruction with intracytoplasmic accumulation of desmin-reactive deposits in cardiac and skeletal muscle cells.
Alternate Names: DES, Desmin
Application Notes: E: 1:500-1:1,000; WB: 1:100-1:500; IHC: 1:100-1:500
Accession No.: P17661
Antigen: Desmin from pig stomach.
Format: Each vial contains 0.1 mg IgG in 0.1 ml (1 mg/ml) of PBS pH7.4, 2% BSA with 0.09% sodium azide. Antibody was purified by Protein-G affinity chromatography.
Storage:
Store at -20°C. Minimize freeze-thaw cycles. Product is guaranteed one year from the date of shipment.
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