Rabbit Desmin Antibody | anti-DES antibody

Desmin Antibody

Cat. Num. AAA8504268

• Gene Names: DES; CSM1; CSM2; CDCD3; CMD1F; LGMD1D; LGMD1E; LGMD2R
• Reactivity: Human, Mouse, Rat
• Applications: Western Blot, Immunohistochemistry
• Purity: Affinity purification
• Synonyms: Desmin; Desmin Antibody; CMD1I; CSM1; CSM2; FLJ12025; FLJ39719; FLJ41013; FLJ41793; anti-DES antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Isotype: IgG
• Purity/Purification: Affinity purification
• Sequence Length: 470

• Applicable Applications for anti-DES antibody: Western Blot (WB), Immunohistochemistry (IHC)
• Application Notes: WB: 1:500-1:1000; IHC: 1:50-1:100
• Immunogen: Fusion protein of human DES
• Storage Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
• Preparation and Storage: Store at-20 degree C or-80 degree C. Avoid freeze/thaw cycles.

Western Blot (WB)

(Western blot analysis of extracts of mouse hearttissue, using DES antibody.)

Related Product Information for anti-DES antibody

The cytoskeleton consists of three types of cytosolic fibers: microfilaments (actin filaments), intermediate filaments and microtubules. Major types of intermediate filaments are distinguished and expressed in particular cell types: cytokeratins (epithelial cells), glial fibrillary acidic protein or GFAP (glial cells), desmin (skeletal, visceral and certain vascular smooth muscle cells), vimentin (mesenchyme origin) and neurofilaments (neurons). GFAP and vimentin form intermediate filaments in astroglial cells and modulate their motility and shape (1). In particular, vimentin filaments are present at early developmental stages, while GFAP filaments are characteristic of differentiated and mature brain astrocytes. Thus, GFAP is commonly used as a marker for intracranial and intraspinal tumors arising from astrocytes (2). Vimentin is present in sarcomas, but not carcinomas, and its expression is examined relative to other markers to distinguish between the two forms of neoplasm (3). Desmin is a myogenic marker expressed in early development that forms a network of filaments that extends across the myofibril and surrounds Z discs. The desmin cytoskeleton provides a connection among myofibrils, organelles and the cytoskeleton (4). Desmin knockout mice develop cardiomyopathy, skeletal and smooth muscle defects (5). In humans, desmin related myopathies might be caused by mutations in the corresponding desmin gene or in proteins with which desmin interacts, including alphaB-crystallin and synemin. Disorganized desmin filaments and the accumulation of protein aggregates comprised predominantly of desmin characterize desmin-related myopathies (reviewed in 6, 7).

Product Categories/Family for anti-DES antibody

Autophagy antibody; Cancer; Cardiovascular; Cell Biology; Epigenetics & Nuclear Signaling; Developmental Biologys; Immunology; Drug Discovery Products; Metabolism; Neuroscience; Signal Transduction; Stem Cells

References

1. Eng, L.F. et al. (2000) Neurochem Res 25, 1439-51. 2. Goebel, H.H. et al. (1987) Acta Histochem Suppl 34, 81-93. 3. Leader, M. et al. (1987) Histopathology 11, 63-72. 4. Capetanaki, Y. et al. (2007) Exp Cell Res 313, 2063-76. 5. Li, Z. et al. (1996) Dev Biol 175, 362-6. 6. Paulin, D. and Li, Z. (2004) Exp Cell Res 301, 1-7. 7. Paulin, D. et al. (2004) J Pathol 204, 418-27.

NCBI and Uniprot Product Information

• NCBI GI #: 55749932
• NCBI GeneID: 1674
• NCBI Accession #: NP_001918.3
• NCBI GenBank Nucleotide #: NM_001927.3
• UniProt Accession #: P17661; Q15787; Q549R7; Q549R8; Q549R9; Q8IZR1; Q8IZR6; Q8NES2; Q8NEU6; Q8TAC4; Q8TCX2; Q8TD99
• Molecular Weight: 54kDa
• NCBI Official Full Name: desmin
• NCBI Official Synonym Full Names: desmin
• NCBI Official Symbol: DES
• NCBI Official Synonym Symbols: CSM1; CSM2; CDCD3; CMD1F; LGMD1D; LGMD1E; LGMD2R
• NCBI Protein Information: desmin
• UniProt Protein Name: Desmin
• Protein Family: Desmin
• UniProt Gene Name: DES

NCBI Description

This gene encodes a muscle-specific class III intermediate filament. Homopolymers of this protein form a stable intracytoplasmic filamentous network connecting myofibrils to each other and to the plasma membrane. Mutations in this gene are associated with desmin-related myopathy, a familial cardiac and skeletal myopathy (CSM), and with distal myopathies. [provided by RefSeq, Jul 2008]

Uniprot Description

Muscle-specific type III intermediate filament essential for proper muscular structure and function. Plays a crucial role in maintaining the structure of sarcomeres, inter-connecting the Z-disks and forming the myofibrils, linking them not only to the sarcolemmal cytoskeleton, but also to the nucleus and mitochondria, thus providing strength for the muscle fiber during activity (PubMed:25358400). 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. Contributes to the transcriptional regulation of the NKX2-5 gene in cardiac progenitor cells during a short period of cardiomyogenesis and in cardiac side population stem cells in the adult. Plays a role in maintaining an optimal conformation of nebulette (NEB) on heart muscle sarcomeres to bind and recruit cardiac alpha-actin ().