Recombinant Human Telethonin(TCAP)

Code CSB-EP023264HU
Size US$2466
Image
  • (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.

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Product Details

Purity Greater than 90% as determined by SDS-PAGE.
Target Names TCAP
Uniprot No. O15273
Research Area Cardiovascular
Alternative Names 19 kDa sarcomeric protein; CMD1N; CMH25; LGMD2G; Limb girdle muscular dystrophy 2G (autosomal recessive); T-cap; Tcap; TELE; Telethonin; TELT_HUMAN; Titin cap protein; Titin-cap
Species Homo sapiens (Human)
Source E.coli
Expression Region 1-167aa
Target Protein Sequence MATSELSCEVSEENCERREAFWAEWKDLTLSTRPEEGCSLHEEDTQRHETYHQQGQCQVLVQRSPWLMMRMGILGRGLQEYQLPYQRVLPLPIFTPAKMGATKEEREDTPIQLQELLALETALGGQCVDRQEVAEITKQLPPVVPVSKPGALRRSLSRSMSQEAQRG
Note: The complete sequence including tag sequence, target protein sequence and linker sequence could be provided upon request.
Mol. Weight 46.1kDa
Protein Length Full Length
Tag Info N-terminal GST-tagged
Form Liquid or Lyophilized powder
Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
Buffer If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol.
Note: If you have any special requirement for the glycerol content, please remark when you place the order.
If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, pH 8.0.
Reconstitution We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20°C/-80°C. Our default final concentration of glycerol is 50%. Customers could use it as reference.
Troubleshooting
and FAQs
Protein FAQs
Storage Condition Store at -20°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
Shelf Life The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
Lead Time Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
Notes Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet & COA Please contact us to get it.

Target Data

Function Muscle assembly regulating factor. Mediates the antiparallel assembly of titin (TTN) molecules at the sarcomeric Z-disk.
Gene References into Functions
  1. based on the crystal structure in which Z1Z2 and telethonin1-90 assemble into a 2:1 complex, a single chain fusion protein was designed, comprising two Z1Z2 modules that are connected by flexible linkers N- and C-terminally of the telethonin1-90. Expression of this fusion protein, named ZTZ, affords high yields of soluble expressed and purified protein PMID: 28811266
  2. Data indicate LGMD2G protein TCAP association with limb girdle muscular dystrophy 2G (LGMD2G). PMID: 25055047
  3. Report TCAP mutations in dilated cardiomyopathy. PMID: 24037902
  4. cardiac telethonin is constitutively bis-phosphorylated and suggest that such phosphorylation is critical for normal telethonin function, which may include maintenance of transverse tubule organization and intracellular Ca(2+) transients. PMID: 24280220
  5. This study identified no pathogenic mutations in BAG3, MATR3, PTRF or TCAP in Australian muscular dystrophy. PMID: 21683594
  6. reduced expression of dystrophin and titin is associated with the pathophysiology of dilated cardiomyopathy, and TNF-alpha may modulate the expression of these proteins via NF-kappaB pathway. PMID: 20373002
  7. Genetic studies of tcap in zebrafish suggested that pathogenesis in LGMD2G is due to a disruption of sarcomere-T-tubular interaction, but not of sarcomere assembly per se. PMID: 19679566
  8. review of telethonin and other new proteins of the Z-disc of skeletal muscle PMID: 11699871
  9. telethonin protein levels seems to be at least in part regulated by neuronal activity and is thus linked to the dynamic control of myofibrillogenesis and muscle turnover in human skeletal muscle. PMID: 11763198
  10. telethonin may play a role in linking titin filaments at the Z-disk periphery PMID: 12446666
  11. TCAP mutations identified which are associated with hypertrophic cardiomyopathy. PMID: 15582318
  12. The titin Z1Z2-telethonin complex resist considerable mechanical force through beta strand crosslinking, suggesting that telethonin is an important component of the N-terminal titin anchor. PMID: 16531234
  13. A dimer of two titin/telethonin complexes is formed within the crystal environment, potentially indicating the formation of higher oligomers. PMID: 16713295
  14. Hypertension induced expression of prohypertrophic BMP10, and the hypertrophic effect of BMP10 was modulated, at least in part, by its binding to Tcap at the Z disc. PMID: 17921333
  15. telethonin is a sodium channel-interacting protein, and its mutations can alter Na(v)1.5 kinetics and may play a role in intestinal pseudo-obstruction PMID: 18408010
  16. Telethonin might be involved in CVB3-mediated cell damage and in the resulting cardiac dysfunction due to the interaction with Siva. PMID: 18849585
  17. Oncogenomic recombination hotspot around the PPP1R1B-STARD3-TCAP-PNMT-PERLD1-ERBB2-C17orf37-GRB7 amplicon at human chromosome 17q12 is closely linked to evolutionary recombination hotspot around the GSDML-GSDM locus. PMID: 15010812

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Involvement in disease Cardiomyopathy, familial hypertrophic 25 (CMH25); Limb-girdle muscular dystrophy 2G (LGMD2G)
Subcellular Location Cytoplasm, myofibril, sarcomere
Tissue Specificity Heart and skeletal muscle.
Database Links

HGNC: 11610

OMIM: 601954

KEGG: hsa:8557

STRING: 9606.ENSP00000312624

UniGene: Hs.77628

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