Desmin (DES) (C-Term) antibody

Details for Product No. ABIN350288
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Antigen
Synonyms DES, des-b, MGC80853, des, csm1, csm2, desm, cmd1i, MGC75911, LOC100220724, desmin, cb290, fb59a12, wu:fb59a12, zgc:109859, CSM1, CSM2, LGMD2R, wu:fc11d08, zgc:154009, des-a, MGC52614
Epitope
C-Term
(45), (13), (12), (12), (8), (7), (6), (3), (2), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1)
Reactivity
Mouse (Murine), Human, Rat (Rattus)
(247), (92), (90), (43), (34), (27), (20), (9), (7), (3), (2), (1), (1), (1)
Host
Rabbit
(170), (89), (10)
Clonality
Polyclonal
Conjugate
Un-conjugated
(5), (5), (5), (3), (3), (3), (3), (3), (3), (3), (3), (2), (2), (2), (1), (1)
Application
Immunohistochemistry (IHC), Western Blotting (WB)
(184), (111), (95), (71), (47), (30), (21), (20), (19), (12), (10), (4), (3), (2), (2), (1), (1), (1)
Pubmed 5 references available
Quantity 1000 μg
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Catalog No. ABIN350288
454.67 $
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Immunogen A synthetic peptide from c-terminal region of mouse Desmin conjugated to an immunogenic carrier protein was used as the antigen. The antigen is homologous in human and rat.
Isotype IgG
Specificity Specific for Desmin.
Alternative Name Desmin
Background 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. Defects in DES are the cause of desmin-related cardio-skeletal myopathy (CSM), also known as desmin-related myopathy (DRM). CSM is characterized by skeletal muscle weakness associated with cardiac conduction blocks, arrhythmias, restrictive heart failure, and by intracytoplasmic accumulation of desmin-reactive deposits in cardiac and skeletal muscle cells. A desmin-related myopathy can have a distal onset, it is then known as hereditary distal myopathy (HDM). Defects in DES are the cause of neurogenic scapuloperoneal syndrome Kaeser type (Kaeser syndrome).
Subcellular location: Cytoplasm. Also known as: Des.
Research Area Cancer, Cytoskeleton
Application Notes A concentration of 10-50 µg/ml is recommended.
The optimal concentration should be determined by the end user.
Not yet tested in other applications.
Restrictions For Research Use only
Format Lyophilized
Reconstitution Reconstitute in 150 µL of sterile water. Centrifuge to remove any insoluble material.
Handling Advice Avoid freeze and thaw cycles.
Storage 4 °C/-20 °C
Storage Comment Maintain the lyophilised/reconstituted antibodies frozen at -20°C for long term storage and refrigerated at 2-8°C for a shorter term. When reconstituting, glycerol (1:1) may be added for an additional stability. Avoid freeze and thaw cycles.
Expiry Date 12 months
General Goudeau, Dagvadorj, Rodrigues-Lima et al.: "Structural and functional analysis of a new desmin variant causing desmin-related myopathy." in: Human mutation, Vol. 18, Issue 5, pp. 388-96, 2001 (PubMed).

Goldfarb, Park, Cervenáková et al.: "Missense mutations in desmin associated with familial cardiac and skeletal myopathy." in: Nature genetics, Vol. 19, Issue 4, pp. 402-3, 1998 (PubMed).

van Groningen, Bloemers, Swart: "Rat desmin gene structure and expression." in: Biochimica et biophysica acta, Vol. 1217, Issue 1, pp. 107-9, 1994 (PubMed).

Li, Choudhary, Milner et al.: "Inhibition of desmin expression blocks myoblast fusion and interferes with the myogenic regulators MyoD and myogenin." in: The Journal of cell biology, Vol. 124, Issue 5, pp. 827-41, 1994 (PubMed).

Li, Lilienbaum, Butler-Browne et al.: "Human desmin-coding gene: complete nucleotide sequence, characterization and regulation of expression during myogenesis and development." in: Gene, Vol. 78, Issue 2, pp. 243-54, 1989 (PubMed).

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