Desmin (clone 10H7D2), anti-human
€377.00
In stock
SKU
252674
Catalog Nr.: 252674
Size: 0.1 mg
Isotype: Mouse IgG1
Applications: WB, IHC
Size: 0.1 mg
Isotype: Mouse IgG1
Applications: 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: Desmin, DES
Application Notes: WB: 1:500-1:2000; IHC: 1:200-1:1000
Accession No.: P17661
Antigen: Purified recombinant fragment of Desmin expressed in E. coli.
Format: Each vial contains 0.1 mg IgG in PBS pH7.4 with 0.03% sodium azide. Antibody was purified by Protein-G affinity chromatography.
Storage:
Store at 4°C for short term use only. Store at -20°C for storage over 1 month. Product is guaranteed 6 months 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: Desmin, DES
Application Notes: WB: 1:500-1:2000; IHC: 1:200-1:1000
Accession No.: P17661
Antigen: Purified recombinant fragment of Desmin expressed in E. coli.
Format: Each vial contains 0.1 mg IgG in PBS pH7.4 with 0.03% sodium azide. Antibody was purified by Protein-G affinity chromatography.
Storage:
Store at 4°C for short term use only. Store at -20°C for storage over 1 month. Product is guaranteed 6 months from the date of shipment.
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