Testing Data
Rabbit Desmin (Paired T76/77) Antibody | anti-Desmin (Paired T76/77) antibody
Rabbit anti Desmin (Paired T76/77), Non-phospho
ELISA: 0.01-0.1 ug/ml
Immunoprecipitation: 2-5 ug/ml
Immunohistochemistry: 2-5 ug/ml
Testing Data
Testing Data
NCBI and Uniprot Product Information
NCBI Description
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]
Uniprot Description
Function: Desmin are class-III intermediate 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.
Subunit structure: Homopolymer. Interacts with DST
By similarity. Interacts with MTM1. Ref.10
Subcellular location: Cytoplasm.
Involvement in disease: Defects in DES are the cause of myopathy myofibrillar desmin-related (MFM-DES) [
MIM:601419]; also known as desmin-related myopathy (DRM). A neuromuscular disorder characterized by skeletal muscle weakness associated with cardiac conduction blocks, arrhythmias, restrictive heart failure, and by myofibrillar destruction with intracytoplasmic accumulation of desmin-reactive deposits in cardiac and skeletal muscle cells. Ref.4 Ref.6 Ref.7 Ref.9 Ref.13 Ref.14 Ref.15 Ref.16 Ref.17 Ref.18 Ref.19 Ref.20 Ref.22Defects in DES are the cause of cardiomyopathy dilated type 1I (CMD1I) [
MIM:604765]. Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death. Ref.5Defects in DES are the cause of neurogenic scapuloperoneal syndrome Kaeser type (Kaeser syndrome) [
MIM:181400]. Kaeser syndrome is an autosomal dominant disorder with a peculiar scapuloperoneal distribution of weakness and atrophy. A large clinical variability is observed ranging from scapuloperoneal, limb grindle and distal phenotypes with variable cardiac or respiratory involvement. Facial weakness, dysphagia and gynaecomastia are frequent additional symptoms. Affected men seemingly bear a higher risk of sudden, cardiac death as compared to affected women. Histological and immunohistochemical examination of muscle biopsy specimens reveal a wide spectrum of findings ranging from near normal or unspecific pathology to typical, myofibrillar changes with accumulation of desmin.
Sequence similarities: Belongs to the intermediate filament family.
Research Articles on Desmin (Paired T76/77)
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Product Notes
The Desmin (Paired T76/77) des (Catalog #AAA462177) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Rabbit anti Desmin (Paired T76/77), Non-phospho reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's Desmin (Paired T76/77) can be used in a range of immunoassay formats including, but not limited to, ELISA, Western Blot, Immunohistochemistry. Western Blot: 0.1-1 ug/ml ELISA: 0.01-0.1 ug/ml Immunoprecipitation: 2-5 ug/ml Immunohistochemistry: 2-5 ug/ml. Researchers should empirically determine the suitability of the Desmin (Paired T76/77) des for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Desmin (Paired T76/77), Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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