Desmin Antibody

Cat no: 251398

Supplier: ABBIOTEC

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Description
Additional Information

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 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. Defects in desmin are the cause of cardiomyopathy dilated type 1I (CMD1I). Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.

Catalogue number:	251398
Reactivities:	Human, Mouse, Rabbit
Applications:	Immunohistochemistry
Size:	0.1 mg
Accession:	P17661
Antigen:	KLH-conjugated synthetic peptide encompassing a sequence within the C-term region of human desmin.
Format:	Each vial contains 0.1 mg IgG in 0.1 ml (1 mg/ml) of PBS pH7.4 with 0.09% sodium azide. Antibody was purified by Protein-G affinity chromatography.
P type:	Rabbit Polyclonal Antibody
Isotype:	Rabbit Ig
Additional info:	DES; Desmin