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Results: 1 to 20 of 68

1.

Proteolytic cleavage of Ser52Pro variant transthyretin triggers its amyloid fibrillogenesis.

Mangione PP, Porcari R, Gillmore JD, Pucci P, Monti M, Porcari M, Giorgetti S, Marchese L, Raimondi S, Serpell LC, Chen W, Relini A, Marcoux J, Clatworthy IR, Taylor GW, Tennent GA, Robinson CV, Hawkins PN, Stoppini M, Wood SP, Pepys MB, Bellotti V.

Proc Natl Acad Sci U S A. 2014 Jan 28;111(4):1539-44. doi: 10.1073/pnas.1317488111. Epub 2014 Jan 13.

PMID:
24474780
[PubMed - indexed for MEDLINE]
Free PMC Article
2.

Repurposing diflunisal for familial amyloid polyneuropathy: a randomized clinical trial.

Berk JL, Suhr OB, Obici L, Sekijima Y, Zeldenrust SR, Yamashita T, Heneghan MA, Gorevic PD, Litchy WJ, Wiesman JF, Nordh E, Corato M, Lozza A, Cortese A, Robinson-Papp J, Colton T, Rybin DV, Bisbee AB, Ando Y, Ikeda S, Seldin DC, Merlini G, Skinner M, Kelly JW, Dyck PJ; Diflunisal Trial Consortium.

JAMA. 2013 Dec 25;310(24):2658-67. doi: 10.1001/jama.2013.283815.

PMID:
24368466
[PubMed - indexed for MEDLINE]
Free PMC Article
3.

Dual Functional Small Molecule Probes as Fluorophore and Ligand for Misfolding Proteins.

Zhang X, Ran C.

Curr Org Chem. 2013 Mar 1;17(6). doi: 10.2174/1385272811317060004.

PMID:
24363605
[PubMed]
Free PMC Article
4.

Effects of tafamidis on transthyretin stabilization and clinical outcomes in patients with non-Val30Met transthyretin amyloidosis.

Merlini G, Planté-Bordeneuve V, Judge DP, Schmidt H, Obici L, Perlini S, Packman J, Tripp T, Grogan DR.

J Cardiovasc Transl Res. 2013 Dec;6(6):1011-20. doi: 10.1007/s12265-013-9512-x. Epub 2013 Oct 8.

PMID:
24101373
[PubMed - indexed for MEDLINE]
Free PMC Article
5.

Transthyretin is dysregulated in preeclampsia, and its native form prevents the onset of disease in a preclinical mouse model.

Kalkunte SS, Neubeck S, Norris WE, Cheng SB, Kostadinov S, Vu Hoang D, Ahmed A, von Eggeling F, Shaikh Z, Padbury J, Berg G, Olofsson A, Markert UR, Sharma S.

Am J Pathol. 2013 Nov;183(5):1425-36. doi: 10.1016/j.ajpath.2013.07.022. Epub 2013 Sep 10.

PMID:
24035612
[PubMed - indexed for MEDLINE]
Free PMC Article
6.

Bifunctional coumarin derivatives that inhibit transthyretin amyloidogenesis and serve as fluorescent transthyretin folding sensors.

Myung N, Connelly S, Kim B, Park SJ, Wilson IA, Kelly JW, Choi S.

Chem Commun (Camb). 2013 Oct 14;49(80):9188-90. doi: 10.1039/c3cc44667k. Epub 2013 Aug 29.

PMID:
23989101
[PubMed - indexed for MEDLINE]
Free PMC Article
7.

Long-term effects of tafamidis for the treatment of transthyretin familial amyloid polyneuropathy.

Coelho T, Maia LF, da Silva AM, Cruz MW, Planté-Bordeneuve V, Suhr OB, Conceiçao I, Schmidt HH, Trigo P, Kelly JW, Labaudinière R, Chan J, Packman J, Grogan DR.

J Neurol. 2013 Nov;260(11):2802-14. doi: 10.1007/s00415-013-7051-7. Epub 2013 Aug 22.

PMID:
23974642
[PubMed - indexed for MEDLINE]
Free PMC Article
8.

NMR paves the way for atomic level descriptions of sparsely populated, transiently formed biomolecular conformers.

Sekhar A, Kay LE.

Proc Natl Acad Sci U S A. 2013 Aug 6;110(32):12867-74. doi: 10.1073/pnas.1305688110. Epub 2013 Jul 18.

PMID:
23868852
[PubMed - indexed for MEDLINE]
Free PMC Article
9.

Familial amyloidotic polyneuropathy: current and emerging treatment options for transthyretin-mediated amyloidosis.

Hund E.

Appl Clin Genet. 2012 Jun 18;5:37-41. doi: 10.2147/TACG.S19903. Print 2012.

PMID:
23776379
[PubMed]
Free PMC Article
10.

Folding the proteome.

Braselmann E, Chaney JL, Clark PL.

Trends Biochem Sci. 2013 Jul;38(7):337-44. doi: 10.1016/j.tibs.2013.05.001. Epub 2013 Jun 11. Review.

PMID:
23764454
[PubMed - indexed for MEDLINE]
Free PMC Article
11.

AG10 inhibits amyloidogenesis and cellular toxicity of the familial amyloid cardiomyopathy-associated V122I transthyretin.

Penchala SC, Connelly S, Wang Y, Park MS, Zhao L, Baranczak A, Rappley I, Vogel H, Liedtke M, Witteles RM, Powers ET, Reixach N, Chan WK, Wilson IA, Kelly JW, Graef IA, Alhamadsheh MM.

Proc Natl Acad Sci U S A. 2013 Jun 11;110(24):9992-7. doi: 10.1073/pnas.1300761110. Epub 2013 May 28.

PMID:
23716704
[PubMed - indexed for MEDLINE]
Free PMC Article
12.

Stress-independent activation of XBP1s and/or ATF6 reveals three functionally diverse ER proteostasis environments.

Shoulders MD, Ryno LM, Genereux JC, Moresco JJ, Tu PG, Wu C, Yates JR 3rd, Su AI, Kelly JW, Wiseman RL.

Cell Rep. 2013 Apr 25;3(4):1279-92. doi: 10.1016/j.celrep.2013.03.024. Epub 2013 Apr 11.

PMID:
23583182
[PubMed - indexed for MEDLINE]
Free PMC Article
13.

Aromatic sulfonyl fluorides covalently kinetically stabilize transthyretin to prevent amyloidogenesis while affording a fluorescent conjugate.

Grimster NP, Connelly S, Baranczak A, Dong J, Krasnova LB, Sharpless KB, Powers ET, Wilson IA, Kelly JW.

J Am Chem Soc. 2013 Apr 17;135(15):5656-68. doi: 10.1021/ja311729d. Epub 2013 Feb 14.

PMID:
23350654
[PubMed - indexed for MEDLINE]
Free PMC Article
14.

Localized structural fluctuations promote amyloidogenic conformations in transthyretin.

Lim KH, Dyson HJ, Kelly JW, Wright PE.

J Mol Biol. 2013 Mar 25;425(6):977-88. doi: 10.1016/j.jmb.2013.01.008. Epub 2013 Jan 11.

PMID:
23318953
[PubMed - indexed for MEDLINE]
Free PMC Article
15.

Expanding the number of 'druggable' targets: non-enzymes and protein-protein interactions.

Makley LN, Gestwicki JE.

Chem Biol Drug Des. 2013 Jan;81(1):22-32. doi: 10.1111/cbdd.12066. Review.

PMID:
23253128
[PubMed - indexed for MEDLINE]
Free PMC Article
16.

True and apparent inhibition of amyloid fibril formation.

Martins PM.

Prion. 2013 Mar-Apr;7(2):136-9. doi: 10.4161/pri.23111. Epub 2012 Dec 11.

PMID:
23232498
[PubMed - indexed for MEDLINE]
Free PMC Article
17.

Transthyretin (TTR) cardiac amyloidosis.

Ruberg FL, Berk JL.

Circulation. 2012 Sep 4;126(10):1286-300. doi: 10.1161/CIRCULATIONAHA.111.078915. Review. No abstract available.

PMID:
22949539
[PubMed - indexed for MEDLINE]
Free PMC Article
18.

Tafamidis for transthyretin familial amyloid polyneuropathy: a randomized, controlled trial.

Coelho T, Maia LF, Martins da Silva A, Waddington Cruz M, Planté-Bordeneuve V, Lozeron P, Suhr OB, Campistol JM, Conceição IM, Schmidt HH, Trigo P, Kelly JW, Labaudinière R, Chan J, Packman J, Wilson A, Grogan DR.

Neurology. 2012 Aug 21;79(8):785-92. doi: 10.1212/WNL.0b013e3182661eb1. Epub 2012 Jul 25.

PMID:
22843282
[PubMed - indexed for MEDLINE]
Free PMC Article
19.

Diflunisal for ATTR cardiac amyloidosis.

Castaño A, Helmke S, Alvarez J, Delisle S, Maurer MS.

Congest Heart Fail. 2012 Nov-Dec;18(6):315-9. doi: 10.1111/j.1751-7133.2012.00303.x. Epub 2012 Jul 2.

PMID:
22747647
[PubMed - indexed for MEDLINE]
Free PMC Article
20.

Multiple substitutions of methionine 129 in human prion protein reveal its importance in the amyloid fibrillation pathway.

Nyström S, Mishra R, Hornemann S, Aguzzi A, Nilsson KP, Hammarström P.

J Biol Chem. 2012 Jul 27;287(31):25975-84. doi: 10.1074/jbc.M112.372136. Epub 2012 Jun 5.

PMID:
22669942
[PubMed - indexed for MEDLINE]
Free PMC Article

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