Host:Rabbit
Target Protein:Desmin
IR:Immunogen Range:261-360/470
Clonality:Polyclonal
Isotype:IgG
Entrez Gene:1674
Swiss Prot:P17661
Source:KLH conjugated synthetic peptide derived from human Desmin:261-360/470
Purification:affinity purified by Protein A
Storage:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Background:Desmin is a muscle-specific, type III intermediate filament that integrates the sarcolemma, Z disk, and nuclear membrane in sarcomeres and regulates sarcomere architecture. 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. Defects in Desmin are the cause of desmin related cardio skeletal myopathy (CSM) also known as desmin related myopathy (DRM). CSM is characterized by skeletal muscle weakness associated with cardiac conduction blocks, arrhythmias, restrictive heart failure, and by intracytoplasmic accumulation of desmin reactive deposits in cardiac and skeletal muscle cells. A desmin related myopathy can have a distal onset, it is then known as hereditary distal myopathy (HDM). Defects in Desmin are also the cause of dilated cardiomyopathy type 1I (CMD1I). CMD1I is an autosomal form of dilated cardiomyopathy characterized by ventricular dilatation and impaired systolic function. Antidesmin antibodies are useful in identification of tumours of myogenic origin.
Size:50ul
Concentration:1mg/ml
Applications:ELISA(1:5000-10000)
IHC-P(1:100-500)
IHC-F(1:100-500)
Flow-Cyt(1μg/Test)
Cross Reactive Species:Human
Mouse
Rat
Chicken
Dog
Pig
Cow
Horse
Rabbit
Sheep
Guinea Pig
本產(chǎn)品僅供科研使用。不能用于人和動物治療等其它臨床診斷使用！
關(guān)鍵字： DES;DESM_HUMAN;Desmin;DES;DESM_HUMAN;
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