Promotional price valid on web orders only. Your contract pricing may differ. Interested in signing up for a dedicated account number?
Learn More

Telethonin Mouse, Unlabeled, Clone: 53, BD

Mouse Monoclonal Antibody

Manufacturer:  BD Biosciences 612328

Catalog No. BDB612328


Add to cart

Description

Description

Autosomal recessive limb-girdle muscular dystrophy (AR LGMD), a genetically heterogeneous group of disorders affecting the proximal musculature, has eight distinct forms: 2A to 2H. Each of these forms is caused by specific gene mutations. A mutation in the telethonin gene causes LGMD 2G. Telethonin, a 19kDa sarcomeric protein found in striated and cardiac muscle, has a developmentally and functionally regulated expression pattern. Telethonin transcript down-regulation occurs in response to muscle denervation. Protein levels of telethonin are partially regulated by neuronal activity, thereby linking telethonin to dynamic control of myofibrillogenesis and muscle turnover in human skeletal muscle. The telethonin transcript exhibits a pattern of accumulation typical of contractile proteins, suggesting a role for the protein in myofibrillar assembly. Immunofluorescence images show that telethonin co-localizes with myosin, which is an expression pattern typical of sarcomeric proteins. Telethonin has a known sarcomeric binding partner, titin, and is also known as titin CAP (TCAP). Therefore, telethonin plays a major role in AR LGMD 2G and is also important as a developmentally and functionally regulated sarcomeric protein.

Western Blotting

Specifications

Specifications

Telethonin
53
Unconjugated
Affinity Purified
Mouse Telethonin aa. 1-167
50μg
Cell Biology
Primary
Human, Murine, Rat
Western Blot
250μg/mL
Aqueous buffered solution containing BSA, glycerol, and ≤0.09% sodium azide.
Murine
IgG1
RUO
Store undiluted at -20°C.
Monoclonal
Documents
Provide Content Correction

We continue to work to improve your shopping experience and your feedback regarding this content is very important to us. Please use the form below to provide feedback related to the content on this product.

Product Title

By clicking Submit, you acknowledge that you may be contacted by Fisher Scientific in regards to the feedback you have provided in this form. We will not share your information for any other purposes. All contact information provided shall also be maintained in accordance with our Privacy Policy.

Cancel Submit