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Anti-Desmin Antibody

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ABclonal
A10838
Overview
Name: Anti-Desmin Antibody
Description: Mouse monoclonal antibody to Desmin.
Applications: WB, IHC, IF, FC
Dilutions: WB 1:500 - 1:2000, IF 1:50 - 1:200
Reactivity: Human
Immunogen: Recombinant protein of human DES
Host: Mouse
Clonality: Monoclonal
Isotype: IgG
Purification: Affinity purification
Formulation: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Storage: Store at -20℃. Avoid freeze / thaw cycles.

Target (Information from UniProt)
Function: Desmin are class-III intermediate filaments found in muscle cells. In adult striated muscle they form a fibrous network connecting myofibrils to each other and to the plasma membrane from the periphery of the Z-line structures (PubMed:24200904, PubMed:25394388, PubMed:26724190). May act as a sarcomeric microtubule-anchoring protein: specifically associates with detyrosinated tubulin-alpha chains, leading to buckled microtubules and mechanical resistance to contraction.
Involvement in Disease: Myopathy, myofibrillar, 1: A form of myofibrillar myopathy, a group of chronic neuromuscular disorders characterized at ultrastructural level by disintegration of the sarcomeric Z disc and myofibrils, and replacement of the normal myofibrillar markings by small dense granules, or larger hyaline masses, or amorphous material. MFM1 is characterized by skeletal muscle weakness associated with cardiac conduction blocks, arrhythmias, restrictive heart failure, and accumulation of desmin-reactive deposits in cardiac and skeletal muscle cells.

Cardiomyopathy, dilated 1I: A disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.

Neurogenic scapuloperoneal syndrome Kaeser type: Autosomal dominant disorder with a peculiar scapuloperoneal distribution of weakness and atrophy. A large clinical variability is observed ranging from scapuloperoneal, limb grindle and distal phenotypes with variable cardiac or respiratory involvement. Facial weakness, dysphagia and gynaecomastia are frequent additional symptoms. Affected men seemingly bear a higher risk of sudden, cardiac death as compared to affected women. Histological and immunohistochemical examination of muscle biopsy specimens reveal a wide spectrum of findings ranging from near normal or unspecific pathology to typical, myofibrillar changes with accumulation of desmin.

Limb-girdle muscular dystrophy 2R: A form of limb-girdle muscular dystrophy, a disease characterized by proximal weakness, weakness of the hip and shoulder girdles and prominent asymmetrical quadriceps femoris and biceps brachii atrophy.
Sequence Similarities: Belongs to the intermediate filament family.
Post-Translational Modification: ADP-ribosylation prevents ability to form intermediate filaments.
Cellular Location: Cytoplasm > Myofibril > Sarcomere > Z line. Cytoplasm. Cell membrane > Sarcolemma.

Localizes in the intercalated disks which occur at the Z line of cardiomyocytes (PubMed:24200904, PubMed:26724190).
UniProt: P17661
Gene ID: 1674
Synonyms: CMD1I Antibody
CSM1 Antibody
CSM2 Antibody
DES Antibody
Desmin (phospho S60) Antibody
DESM_HUMAN Antibody
FLJ12025 Antibody
FLJ39719 Antibody
FLJ41013 Antibody
FLJ41793 Antibody
Intermediate filament protein Antibody
OTTHUMP00000064865 Antibody

Images
Western Blot - Anti-Desmin Antibody from ABclonal (A10838) - Antibody Supply Service
Western blot analysis of extracts of HeLa cells, using DES antibody.

Product Code - A10838
Size Price Quantity
100µl £307
200µl £452

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