Desmin (DES) (C-Term) antibody

Antigen: Desmin (DES)

Synonyms: DES, des-a, MGC52614, des-b, MGC80853, CSM1, CSM2, CMD1I, FLJ12025, FLJ39719, FLJ41013, FLJ41793, des, csm1, csm2, desm, cmd1i, MGC75911, LOC395906, LOC100220724, desmin, cb290, fb59a12, MGC109859, wu:fb59a12, zgc:109859, MGC154009, wu:fc11d08

Epitope: C-Term

Clonality: Polyclonal

Host: Rabbit

Application: Immunohistochemistry (IHC), Western Blotting (WB)

Catalog no.: ABIN350287

Additional Information

Alternative name: Desmin (c-terminal region)

Swiss-Prot: P31001

Immunogen: A synthetic peptide from c-terminal region of mouse Desmin conjugated to an immunogenic carrier protein was used as the immunogen. The antigen is homologous in human and rat.

Format: Lyophilized

Description: 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.

Specificity: Appears to be specific for Desmin.

Synonyms: DES, des-a, MGC52614, des-b, MGC80853, CSM1, CSM2, CMD1I, FLJ12025, FLJ39719, FLJ41013, FLJ41793, des, csm1, csm2, desm, cmd1i, MGC75911, LOC395906, LOC100220724, desmin, cb290, fb59a12, MGC109859, wu:fb59a12, zgc:109859, MGC154009, wu:fc11d08

Application Details

Application Notes: IHC, WB. A dilution of 1 : 300 to 1 : 2000 is recommended. The optimal dilution should be determined by the end user. Not yet tested in other applications.

Purity: whole serum

Storage: 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.

Restrictions: For Research Use only

Publications

Publications: 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).

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).

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

Goldfarb, Park, Cervenuakovua 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).

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).