Product Name	Desmin / PerCP
Chinese Name	PerCP标记的结蛋白抗体
Alias	DESM_HUMAN; DES;
Research Area	Tumour  Cardiovascular  immunology  Signal transduction  Cell type markers
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	(predicted: Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, Guinea Pig, )
Applications	IF=1:20-200  not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	52kDa
Cellular localization	cytoplasmic
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human Desmin: 261-360/470
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	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. SWISS: P17661 Gene ID: 1674 Database links: Entrez Gene: 1674 Human Entrez Gene: 13346 Mouse Entrez Gene: 64362 Rat Omim: 125660 Human SwissProt: P17661 Human SwissProt: P31001 Mouse SwissProt: P48675 Rat Unigene: 594952 Human Unigene: 6712 Mouse Unigene: 39196 Rat

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