Desmin (D93F5) XP? Rabbit mAb

• 产品编号：CST-5332S      品牌：CST       原厂货号：5332S
• 产品分类：抗体 > 一抗 > 蛋白特异性一抗
• 应用分类：

描述：

Desmin (D93F5) XP® Rabbit mAb兔单抗检测内源性desmin总蛋白。通过人工合成人源desmin蛋白羧基末端相应的多肽片段去免疫动物从而制备出单克隆抗体。细胞骨架由三种细胞基质纤维组成：微丝(actin filaments)、中间纤维和微管。中间纤维的主要类型是有区别的，且它在特别的细胞中表达：细胞角蛋白（epithelial细胞）、胶质原纤维酸性蛋白 或GFAP（glial细胞）、desmin （skeletal、viscera和某种vascular smooth muscle 细胞）、波形蛋白（间充质来源）和神经纤维细丝（neurons）。GFAP和vimentin蛋白在星形胶质细胞瘤细胞中形成中间纤维，并且调节它们的活性和形状(1)。尤其，vimentin filaments在早期发育阶段出现，而GFAP filaments是分化的和成熟的大脑星形胶质细胞的特征。因此，GFAP普遍被用作来自星形胶质细胞颅内和椎管内肿瘤上升的标记物 (2)。Vimentin出现在恶性毒瘤中，但不出现癌中，因此相对于其它标记物该蛋白的表达被检测用于区别两种形式的肿瘤(3) 。Desmin蛋白是肌原性的标记物，其表达在早期filaments网络形成的发展期，其恩呢该延伸穿过肌原纤维和Z discs周围。desmin细胞骨架提供了一个在肌原纤维、细胞器和细胞骨架之中的连接(4)。Desmin 基因敲除的小鼠会发展成心肌病、骨骼肌和平滑肌缺陷(5)。在人类，通过在相应的desmin基因或在desmin 相互作用的蛋白包括αB-crystallin 和synemin蛋白的突变，desmin蛋白相关的肌肉疾病可能被引起。紊乱的desmin filaments和蛋白质聚合物主要包含desmin的堆积都是desmin相关的肌肉疾病的特征 (6,7)。

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.

原厂资料：

Homology

Species predicted to react based on 100% sequence homology: Monkey

Specificity / Sensitivity

Desmin (D93F5) XP® Rabbit mAb detects endogenous levels of total desmin protein.

Source / Purification

Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to carboxy terminal residues of human desmin protein.

Background

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 αB-crystallin and synemin. Disorganized desmin filaments and the accumulation of protein aggregates comprised predominantly of desmin characterize desmin-related myopathies (reviewed in 6,7).

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.

注意事项：

Storage: Supplied in 10 mM sodium HEPES (pH 7.5), 150
mM NaCl, 100 µg/ml BSA, 50% glycerol and less than 0.02%
sodium azide. Store at –20°C. Do not aliquot the antibody.