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Desmin Antibody
ABBIOTEC

Supplier:

ABBIOTEC

Cat no: 251398

Desmin Antibody

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.

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SPECIFICATIONS

Catalog Number

251398

Size

0.1 mg

Applications

IHC

Reactivities

Hum, Mouse, Rabbit

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

Antigen

KLH-conjugated synthetic peptide encompassing a sequence within the C-term region of human desmin.

Isotype

Rabbit Ig

Accession

P17661

Additional Info

DES; Desmin

SUPPLIER INFO

ABBIOTEC

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