Titin-Cap (Telethonin) (TCAP) (AA 1-167) antibody

Antigen: Titin-Cap (Telethonin) (TCAP)

Synonyms: TELE, CMD1N, T-cap, LGMD2G, telethonin, TCAP

Binding Site: AA 1-167

Clonality: Monoclonal (53)

Host: Mouse

Reactivity: Mouse (Murine)

Application: Western Blotting (WB)

Catalog no.: ABIN968822

Additional Information

Alternative name: Telethonin

Immunogen: Mouse Telethonin

Cross-Reactivity: Human, Rat (Rattus)

Format: Liquid

Isotype: IgG1

Clone: 53

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 19 kDa 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.

Characteristics: 1. Since applications vary, each investigator should titrate the reagent to obtain optimal results.
2. Please refer to us for technical protocols.
3. Caution: Sodium azide yields highly toxic hydrazoic acid under acidic conditions. Dilute azide compounds in running water before discarding to avoid accumulation of potentially explosive deposits in plumbing.
4. Source of all serum proteins is from USDA inspected abattoirs located in the United States.

Molecular Weight: 19 kDa

Application Details

Concentration: 250 µg/ml

Purity: Purified

Purification: Purified from tissue culture supernatant or ascites by affinity chromatography.

Buffer: Aqueous buffered solution containing BSA, glycerol.

Preservative: 0.09% Sodium azide.

Storage: Store undiluted at -20° C.

Restrictions: For Research Use only

Images

Western blot analysis of Telethonin of mouse heart lysate. Lane 1: 1:1000, lane 2: 1:2000, lane 3: 1:4000 dilution of Telethonin.

Publications

Product: Valle, Faulkner, De Antoni et al.: "Telethonin, a novel sarcomeric protein of heart and skeletal muscle." in: FEBS letters, Vol. 415, Issue 2, pp. 163-8, 1997 (PubMed).

Mason, Bayol, Loughna: "The novel sarcomeric protein telethonin exhibits developmental and functional regulation." in: Biochemical and biophysical research communications, Vol. 257, Issue 3, pp. 699-703, 1999 (PubMed).

Moreira, Wiltshire, Faulkner et al.: "Limb-girdle muscular dystrophy type 2G is caused by mutations in the gene encoding the sarcomeric protein telethonin." in: Nature genetics, Vol. 24, Issue 2, pp. 163-6, 2000 (PubMed).

Schröder, Reimann, Iakovenko et al.: "Early and selective disappearance of telethonin protein from the sarcomere in neurogenic atrophy." in: Journal of muscle research and cell motility, Vol. 22, Issue 3, pp. 259-64, 2001 (PubMed).