ARG66873

anti-Desmin antibody [SQab21245]

anti-Desmin antibody [SQab21245] for IHC-Formalin-fixed paraffin-embedded sections and Human

Overview

Product Description Mouse Monoclonal antibody [SQab21245] recognizes Desmin
Tested Reactivity Hu
Tested Application IHC-P
Host Mouse
Clonality Monoclonal
Clone SQab21245
Isotype IgG
Target Name Desmin
Antigen Species Human
Immunogen Derived from Human Leiomyoma.
Conjugation Un-conjugated
Alternate Names LGMD2R; CSM1; Desmin; CSM2

Application Instructions

Application Suggestion
Tested Application Dilution
IHC-P1:100 - 1:200
Application Note 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.

Properties

Form Liquid
Purification Purification with Protein G.
Buffer PBS, 0.01% Sodium azide, 40% Glycerol and 0.05% BSA.
Preservative 0.01% Sodium azide
Stabilizer 40% Glycerol and 0.05% BSA
Storage Instruction 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 laboratory research only, not for drug, diagnostic or other use.

Bioinformation

Database Links

GeneID: 1674 Human DES

Swiss-port # P17661 Human Desmin

Gene Symbol DES
Gene Full Name desmin
Background 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]
Function 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]
Cellular Localization 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]
Calculated MW 54 kDa
PTM ADP-ribosylation prevents ability to form intermediate filaments. [UniProt]