anti-Desmin antibody [SQab21245]
anti-Desmin antibody [SQab21245] for IHC-Formalin-fixed paraffin-embedded sections and Human
|产品描述||Mouse Monoclonal antibody [SQab21245] recognizes Desmin|
|抗原||Derived from Human Leiomyoma.|
|別名||LGMD2R; CSM1; Desmin; CSM2|
|应用说明||IHC-P: Antigen Retrieval: Heat mediation was performed in Tris/EDTA buffer (pH 9.0).
* The dilutions indicate recommended starting dilutions and the optimal dilutions or concentrations should be determined by the scientist.
|纯化||Purification with Protein G.|
|缓冲液||PBS, 0.01% Sodium azide, 40% Glycerol and 0.05% BSA.|
|抗菌剂||0.01% Sodium azide|
|稳定剂||40% Glycerol and 0.05% BSA|
|存放说明||For continuous use, store undiluted antibody at 2-8°C for up to a week. For long-term storage, aliquot and store at -20°C. Storage in frost free freezers is not recommended. Avoid repeated freeze/thaw cycles. Suggest spin the vial prior to opening. The antibody solution should be gently mixed before use.|
|注意事项||For laboratory research only, not for drug, diagnostic or other use.|
|背景介绍||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]|
|生物功能||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 (By similarity). [UniProt]|
|细胞定位||Cytoplasm, myofibril, sarcomere, Z line. Cytoplasm. Cell membrane, sarcolemma. Nucleus. Note=Localizes in the intercalated disks which occur at the Z line of cardiomyocytes (PubMed:24200904, PubMed:26724190). Localizes in the nucleus exclusively in differentiating cardiac progenitor cells and premature cardiomyocytes (By similarity). [UniProt]|
|翻译后修饰||ADP-ribosylation prevents ability to form intermediate filaments. [UniProt]|
检测图片 (1) Click the Picture to Zoom In
ARG66873 anti-Desmin antibody [SQab21245] IHC-P image
Immunohistochemistry: Formalin-fixed and paraffin-embedded Human esophagus tissue. Antigen Retrieval: Heat mediation was performed in Tris/EDTA buffer (pH 9.0). The tissue section was stained with ARG66873 anti-Desmin antibody [SQab21245] for 30 min at RT.