Transthyretin

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Contents

Function

Transthyretin (TTR) is a serum carrier of the thyroid hormone thyroxine (T4) and retinol through its association with retinol-binding protein (RBP). Many small molecules bind to TTR T4-binding site[1]. For details see Tafamidis and Student Project 2 for UMass Chemistry 423 Spring 2015.

Disease

TTR mutations are associated with amyloid deposition[2]. Tafamidis is a medication which stabilises TTR and is used in treatment of TTR amyloidosis[3].

Structural highlights

The hormone tyrosine is bound in the active site of TTR[4].

3D structures of transthyretin

Transthyretin 3D structures


Human transthyretin complex with tyroxine derivative 1tha

Drag the structure with the mouse to rotate

References

  1. Robbins J. Transthyretin from discovery to now. Clin Chem Lab Med. 2002 Dec;40(12):1183-90. PMID:12553418 doi:http://dx.doi.org/10.1515/CCLM.2002.208
  2. Saraiva MJ. Transthyretin mutations in health and disease. Hum Mutat. 1995;5(3):191-6. PMID:7599630 doi:http://dx.doi.org/10.1002/humu.1380050302
  3. Maurer MS, Schwartz JH, Gundapaneni B, Elliott PM, Merlini G, Waddington-Cruz M, Kristen AV, Grogan M, Witteles R, Damy T, Drachman BM, Shah SJ, Hanna M, Judge DP, Barsdorf AI, Huber P, Patterson TA, Riley S, Schumacher J, Stewart M, Sultan MB, Rapezzi C. Tafamidis Treatment for Patients with Transthyretin Amyloid Cardiomyopathy. N Engl J Med. 2018 Sep 13;379(11):1007-1016. PMID:30145929 doi:10.1056/NEJMoa1805689
  4. Wojtczak A, Luft J, Cody V. Mechanism of molecular recognition. Structural aspects of 3,3'-diiodo-L-thyronine binding to human serum transthyretin. J Biol Chem. 1992 Jan 5;267(1):353-7. PMID:1730601

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Michal Harel, Alexander Berchansky

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