Desmin Polyclonal Antibody, PerCP Conjugated
Product Specifications
Background
Filaments found in muscle cells. 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.
Synonyms
CMD1I; CSM1; CSM2; DES; FLJ12025; FLJ39719; FLJ41013; FLJ41793; Intermediate filament protein; OTTHUMP00000064865; DESM_HUMAN; Desmin; FLJ12025; FLJ39719; FLJ41013; FLJ41793.
Gene ID
1674
Swiss Prot
P17661
Host
Rabbit
Cross Reactivity
Rat
Immunogen
311-400/470
Target
Desmin
Clonality
Polyclonal
Isotype
IgG
Conjugation
PerCP
Source
KLH conjugated synthetic peptide derived from human Desmin
Applications
IF (ICC), IF
Purification
Purified by Protein A.
Excitation Emission
482nm/677nm
Concentration
1µg/µl
Dilution
IF (ICC) (1:50-200), IF ()
Buffer
Aqueous buffered solution containing 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Modification
Unmodified
Storage Conditions
Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
Gene ID URL
1674
Predicted Cross Reactivity
Human, Mouse, Dog, Cow, Sheep, Pig, Horse, Rabbit
Curated Selection
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