General Information
Alternative Names | LGMD2R; CSM1; Desmin; CSM2 |
Target Name | Desmin |
Description | Recombinant rabbit monoclonal antibody recognizes desmin |
Species Reactivity | Hu, Ms, Rat |
Host | Rabbit |
Host Clonality | Rabbit mAb |
Clone | Monoclonal |
Clone Number | SQab1722 |
Isotope | IgG |
Antigen Species | Human |
Antigen | Synthetic peptide around the C-terminus of desmin. |
Conjugate | Un-conjugated |
Application | IHC-P, WB |
Package | 50 μL; 100 μL |
Form | Liquid |
Purification | Affinity purification with immunogen. |
Buffer | PBS, 0.01% Sodium azide, 40% Glycerol and 0.05% BSA |
Storage | 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. |
Note | For research use only. Not intended for any clinical use. |
Application Suggestions
Recommended Dilution for WB | 1:5000 ‐ 1:10000 |
Recommended Dilution for IHC-P | 1:4000 ‐ 1:8000 |
Application Notes | IHC-P: Antigen Retrieval: Heat mediation was performed using Tris/EDTA buffer (pH 9.0) * The dilutions indicate recommended starting dilutions and the optimal dilutions or concentrations should be determined by the scientist. |
Bioinformation
Gene Name | DES |
Gene Full Name | Desmin |
Background | Desmin is 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. |
Biological Functions | Desmin: 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. [UniProt] |
Research Field | Cancer antibody; Cell biology and cellular response antibody; Developmental biology antibody; Signaling transduction antibody; Smooth muscle marker antibody; Cardiomyocyte cell surface marker antibody; Mural cell marker antibody; Microvascular density study antibody |
Predict Molecular Weight | 54 kDa |
Post-Translational Modification | ADP-ribosylation prevents ability to form intermediate filaments. |
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