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EXPLORE: A Prospective, Multinational, Natural History Study of Patients with Acute Hepatic Porphyria with Recurrent Attacks.

Gouya L, Ventura P, Balwani M, Bissell DM, Rees DC, Stölzel U, Phillips JD, Kauppinen R, Langendonk JG, Desnick RJ, Deybach JC, Bonkovsky HL, Parker C, Naik H, Badminton M, Stein PE, Minder E, Windyga J, Bruha R, Cappellini MD, Sardh E, Harper P, Sandberg S, Aarsand AK, Andersen J, Alegre F, Ivanova A, Talbi N, Chan A, Querbes W, Ko J, Penz C, Liu S, Lin T, Simon A, Anderson KE.

Hepatology. 2019 Sep 12. doi: 10.1002/hep.30936. [Epub ahead of print]


Localized Electronic Structure of Nitrogenase FeMoco Revealed by Selenium K-Edge High Resolution X-ray Absorption Spectroscopy.

Henthorn JT, Arias RJ, Koroidov S, Kroll T, Sokaras D, Bergmann U, Rees DC, DeBeer S.

J Am Chem Soc. 2019 Aug 28;141(34):13676-13688. doi: 10.1021/jacs.9b06988. Epub 2019 Aug 15.


Structures of the Neisseria meningitides methionine-binding protein MetQ in substrate-free form and bound to l- and d-methionine isomers.

Nguyen PT, Lai JY, Kaiser JT, Rees DC.

Protein Sci. 2019 Oct;28(10):1750-1757. doi: 10.1002/pro.3694. Epub 2019 Aug 9.


The effects of hydroxycarbamide on the plasma proteome of children with sickle cell anaemia.

Brewin J, Tewari S, Menzel S, Kirkham F, Inusa B, Renney G, Ward M, Rees DC.

Br J Haematol. 2019 Sep;186(6):879-886. doi: 10.1111/bjh.15996. Epub 2019 May 29.


Author Correction: Novel mutations in PIEZO1 cause an autosomal recessive generalized lymphatic dysplasia with non-immune hydrops fetalis.

Fotiou E, Martin-Almedina S, Simpson MA, Lin S, Gordon K, Brice G, Atton G, Jeffery I, Rees DC, Mignot C, Vogt J, Homfray T, Snyder MP, Rockson SG, Jeffery S, Mortimer PS, Mansour S, Ostergaard P.

Nat Commun. 2019 Apr 26;10(1):1951. doi: 10.1038/s41467-019-09905-4.


The effect of the antisickling compound GBT1118 on the permeability of red blood cells from patients with sickle cell anemia.

Al Balushi H, Dufu K, Rees DC, Brewin JN, Hannemann A, Oksenberg D, Lu DC, Gibson JS.

Physiol Rep. 2019 Mar;7(6):e14027. doi: 10.14814/phy2.14027.


Site-Specific Oxidation State Assignments of the Iron Atoms in the [4Fe:4S]2+/1+/0 States of the Nitrogenase Fe-Protein.

Wenke BB, Spatzal T, Rees DC.

Angew Chem Int Ed Engl. 2019 Mar 18;58(12):3894-3897. doi: 10.1002/anie.201813966. Epub 2019 Feb 14.


Structure and belonging: Pathways to success for underrepresented minority and women PhD students in STEM fields.

Fisher AJ, Mendoza-Denton R, Patt C, Young I, Eppig A, Garrell RL, Rees DC, Nelson TW, Richards MA.

PLoS One. 2019 Jan 9;14(1):e0209279. doi: 10.1371/journal.pone.0209279. eCollection 2019.


High body mass index in children with sickle cell disease: a retrospective single-centre audit.

Hall R, Gardner K, Rees DC, Chakravorty S.

BMJ Paediatr Open. 2018 Oct 23;2(1):e000302. doi: 10.1136/bmjpo-2018-000302. eCollection 2018.


Addressing the diagnostic gaps in pyruvate kinase deficiency: Consensus recommendations on the diagnosis of pyruvate kinase deficiency.

Bianchi P, Fermo E, Glader B, Kanno H, Agarwal A, Barcellini W, Eber S, Hoyer JD, Kuter DJ, Maia TM, Mañu-Pereira MDM, Kalfa TA, Pissard S, Segovia JC, van Beers E, Gallagher PG, Rees DC, van Wijk R; with the endorsement of EuroBloodNet, the European Reference Network in Rare Hematological Diseases.

Am J Hematol. 2019 Jan;94(1):149-161. doi: 10.1002/ajh.25325. Epub 2018 Nov 28.


Noncanonical role for the binding protein in substrate uptake by the MetNI methionine ATP Binding Cassette (ABC) transporter.

Nguyen PT, Lai JY, Lee AT, Kaiser JT, Rees DC.

Proc Natl Acad Sci U S A. 2018 Nov 6;115(45):E10596-E10604. doi: 10.1073/pnas.1811003115. Epub 2018 Oct 23.


Elucidating a role for the cytoplasmic domain in the Mycobacterium tuberculosis mechanosensitive channel of large conductance.

Herrera N, Maksaev G, Haswell ES, Rees DC.

Sci Rep. 2018 Oct 1;8(1):14566. doi: 10.1038/s41598-018-32536-6.


A gain of function variant in PIEZO1 (E756del) and sickle cell disease.

Rooks H, Brewin J, Gardner K, Chakravorty S, Menzel S, Hannemann A, Gibson J, Rees DC.

Haematologica. 2019 Mar;104(3):e91-e93. doi: 10.3324/haematol.2018.202697. Epub 2018 Sep 20. No abstract available.


The "speed limit" for macromolecular crystal growth.

Arias RJ, Kaiser JT, Rees DC.

Protein Sci. 2018 Oct;27(10):1837-1841. doi: 10.1002/pro.3491. Epub 2018 Oct 16.


Lipid metabolism in terminal erythropoiesis.

Gibson JS, Rees DC.

Blood. 2018 Jun 28;131(26):2872-2874. doi: 10.1182/blood-2018-05-850255. No abstract available.


Oxidative stress and phosphatidylserine exposure in red cells from patients with sickle cell anaemia.

Hannemann A, Rees DC, Brewin JN, Noe A, Low B, Gibson JS.

Br J Haematol. 2018 Aug;182(4):567-578. doi: 10.1111/bjh.15441. Epub 2018 Jun 25.


A survey of genetic fetal-haemoglobin modifiers in Nigerian patients with sickle cell anaemia.

Adeyemo TA, Ojewunmi OO, Oyetunji IA, Rooks H, Rees DC, Akinsulie AO, Akanmu AS, Thein SL, Menzel S.

PLoS One. 2018 Jun 7;13(6):e0197927. doi: 10.1371/journal.pone.0197927. eCollection 2018.


Fragment-Based Discovery of a Potent, Orally Bioavailable Inhibitor That Modulates the Phosphorylation and Catalytic Activity of ERK1/2.

Heightman TD, Berdini V, Braithwaite H, Buck IM, Cassidy M, Castro J, Courtin A, Day JEH, East C, Fazal L, Graham B, Griffiths-Jones CM, Lyons JF, Martins V, Muench S, Munck JM, Norton D, O'Reilly M, Palmer N, Pathuri P, Reader M, Rees DC, Rich SJ, Richardson C, Saini H, Thompson NT, Wallis NG, Walton H, Wilsher NE, Woolford AJ, Cooke M, Cousin D, Onions S, Shannon J, Watts J, Murray CW.

J Med Chem. 2018 Jun 14;61(11):4978-4992. doi: 10.1021/acs.jmedchem.8b00421. Epub 2018 May 31.


White matter integrity and processing speed in sickle cell anemia.

Stotesbury H, Kirkham FJ, Kölbel M, Balfour P, Clayden JD, Sahota S, Sakaria S, Saunders DE, Howard J, Kesse-Adu R, Inusa B, Pelidis M, Chakravorty S, Rees DC, Awogbade M, Wilkey O, Layton M, Clark CA, Kawadler JM.

Neurology. 2018 Jun 5;90(23):e2042-e2050. doi: 10.1212/WNL.0000000000005644. Epub 2018 May 11.


Organic synthesis provides opportunities to transform drug discovery.

Blakemore DC, Castro L, Churcher I, Rees DC, Thomas AW, Wilson DM, Wood A.

Nat Chem. 2018 Apr;10(4):383-394. doi: 10.1038/s41557-018-0021-z. Epub 2018 Mar 22. Review.


Proteomic analysis of plasma from children with sickle cell anemia and silent cerebral infarction.

Tewari S, Renney G, Brewin J, Gardner K, Kirkham F, Inusa B, Barrett JE, Menzel S, Thein SL, Ward M, Rees DC.

Haematologica. 2018 Jul;103(7):1136-1142. doi: 10.3324/haematol.2018.187815. Epub 2018 Mar 15.


The effect of xanthine oxidase and hypoxanthine on the permeability of red cells from patients with sickle cell anemia.

Al Balushi HWM, Rees DC, Brewin JN, Hannemann A, Gibson JS.

Physiol Rep. 2018 Mar;6(5). doi: 10.14814/phy2.13626.


g(HbF): a genetic model of fetal hemoglobin in sickle cell disease.

Gardner K, Fulford T, Silver N, Rooks H, Angelis N, Allman M, Nkya S, Makani J, Howard J, Kesse-Adu R, Rees DC, Stuart-Smith S, Yeghen T, Awogbade M, Sangeda RZ, Mgaya J, Patel H, Newhouse S, Menzel S, Thein SL.

Blood Adv. 2018 Feb 13;2(3):235-239. doi: 10.1182/bloodadvances.2017009811.


How I manage red cell transfusions in patients with sickle cell disease.

Rees DC, Robinson S, Howard J.

Br J Haematol. 2018 Feb;180(4):607-617. doi: 10.1111/bjh.15115. Epub 2018 Jan 29. Review.


Overnight auto-adjusting continuous airway pressure + standard care compared with standard care alone in the prevention of morbidity in sickle cell disease phase II (POMS2b): study protocol for a randomised controlled trial.

Howard J, Slee AE, Skene S, Inusa B, Kawadler J, Downes M, Gavlak J, Koelbel M, Stotesbury H, Chorozoglou M, Tebbs S, Chakravorty S, Awogbade M, Rees DC, Gupta A, Murphy PB, Hart N, Sahota S, Nwosu C, Gwam M, Saunders D, Muthurangu V, Barber N, Ako E, Thein SL, Marshall M, Reading IC, Cheng MYE, Kirkham FJ, Liossi C.

Trials. 2018 Jan 22;19(1):55. doi: 10.1186/s13063-017-2419-0.


Are the risks of treatment to cure a child with severe sickle cell disease too high?

de Montalembert M, Brousse V, Chakravorty S, Pagliuca A, Porter J, Telfer P, Vora A, Rees DC.

BMJ. 2017 Nov 23;359:j5250. doi: 10.1136/bmj.j5250. No abstract available.


Fragment-to-Lead Medicinal Chemistry Publications in 2016.

Johnson CN, Erlanson DA, Jahnke W, Mortenson PN, Rees DC.

J Med Chem. 2018 Mar 8;61(5):1774-1784. doi: 10.1021/acs.jmedchem.7b01298. Epub 2017 Oct 31. Review.


Correction to Reversible Protonated Resting State of the Nitrogenase Active Site.

Morrison CN, Spatzal T, Rees DC.

J Am Chem Soc. 2017 Oct 4;139(39):13958. doi: 10.1021/jacs.7b09849. Epub 2017 Sep 26. No abstract available.


The significance of inadequate transcranial Doppler studies in children with sickle cell disease.

Greenwood S, Deane C, Rees OL, Freedman B, Kumar S, Ben Ramadan N, Wilkinson S, Marais G, Lord J, Chakravorty S, Height SE, Gardner K, Rees DC.

PLoS One. 2017 Jul 25;12(7):e0181681. doi: 10.1371/journal.pone.0181681. eCollection 2017.


Sickle Cell Disease.

Piel FB, Steinberg MH, Rees DC.

N Engl J Med. 2017 Jul 20;377(3):305. doi: 10.1056/NEJMc1706325. No abstract available.


Electrochemical and structural characterization of Azotobacter vinelandii flavodoxin II.

Segal HM, Spatzal T, Hill MG, Udit AK, Rees DC.

Protein Sci. 2017 Oct;26(10):1984-1993. doi: 10.1002/pro.3236. Epub 2017 Aug 30.


The super sickling haemoglobin HbS-Oman: a study of red cell sickling, K+ permeability and associations with disease severity in patients heterozygous for HbA and HbS-Oman (HbA/S-Oman genotype).

Al Balushi HWM, Wali Y, Al Awadi M, Al-Subhi T, Rees DC, Brewin JN, Hannemann A, Gibson JS.

Br J Haematol. 2017 Oct;179(2):256-265. doi: 10.1111/bjh.14851. Epub 2017 Jul 12.


Reversible Protonated Resting State of the Nitrogenase Active Site.

Morrison CN, Spatzal T, Rees DC.

J Am Chem Soc. 2017 Aug 9;139(31):10856-10862. doi: 10.1021/jacs.7b05695. Epub 2017 Jul 26. Erratum in: J Am Chem Soc. 2017 Oct 4;139(39):13958.


Prasugrel hydrochloride for the treatment of sickle cell disease.

Conran N, Rees DC.

Expert Opin Investig Drugs. 2017 Jul;26(7):865-872. doi: 10.1080/13543784.2017.1335710. Epub 2017 Jun 12. Review.


Recommendations regarding splenectomy in hereditary hemolytic anemias.

Iolascon A, Andolfo I, Barcellini W, Corcione F, Garçon L, De Franceschi L, Pignata C, Graziadei G, Pospisilova D, Rees DC, de Montalembert M, Rivella S, Gambale A, Russo R, Ribeiro L, Vives-Corrons J, Martinez PA, Kattamis A, Gulbis B, Cappellini MD, Roberts I, Tamary H; Working Study Group on Red Cells and Iron of the EHA.

Haematologica. 2017 Aug;102(8):1304-1313. doi: 10.3324/haematol.2016.161166. Epub 2017 May 26.


Sickle Cell Disease.

Piel FB, Steinberg MH, Rees DC.

N Engl J Med. 2017 Apr 20;376(16):1561-1573. doi: 10.1056/NEJMra1510865. Review. No abstract available.


Discovery and Pharmacological Characterization of JNJ-42756493 (Erdafitinib), a Functionally Selective Small-Molecule FGFR Family Inhibitor.

Perera TPS, Jovcheva E, Mevellec L, Vialard J, De Lange D, Verhulst T, Paulussen C, Van De Ven K, King P, Freyne E, Rees DC, Squires M, Saxty G, Page M, Murray CW, Gilissen R, Ward G, Thompson NT, Newell DR, Cheng N, Xie L, Yang J, Platero SJ, Karkera JD, Moy C, Angibaud P, Laquerre S, Lorenzi MV.

Mol Cancer Ther. 2017 Jun;16(6):1010-1020. doi: 10.1158/1535-7163.MCT-16-0589. Epub 2017 Mar 24.


An Audit of the Use of Gonadorelin Analogues to Prevent Recurrent Acute Symptoms in Patients with Acute Porphyria in the United Kingdom.

Schulenburg-Brand D, Gardiner T, Guppy S, Rees DC, Stein P, Barth J, Felicity Stewart M, Badminton M.

JIMD Rep. 2017;36:99-107. doi: 10.1007/8904_2017_2. Epub 2017 Feb 21.


Update review of the acute porphyrias.

Stein PE, Badminton MN, Rees DC.

Br J Haematol. 2017 Feb;176(4):527-538. doi: 10.1111/bjh.14459. Epub 2016 Dec 16. Review.


Real-time dose adjustment using point-of-care platelet reactivity testing in a double-blind study of prasugrel in children with sickle cell anaemia.

Jakubowski JA, Hoppe CC, Zhou C, Smith BE, Brown PB, Heath LE, Inusa B, Rees DC, Small DS, Gupta N, Yao S, Heeney M, Kanter J.

Thromb Haemost. 2017 Feb 28;117(3):580-588. doi: 10.1160/TH16-09-0731. Epub 2016 Dec 8.


Associations between environmental factors and hospital admissions for sickle cell disease.

Piel FB, Tewari S, Brousse V, Analitis A, Font A, Menzel S, Chakravorty S, Thein SL, Inusa B, Telfer P, de Montalembert M, Fuller GW, Katsouyanni K, Rees DC.

Haematologica. 2017 Apr;102(4):666-675. doi: 10.3324/haematol.2016.154245. Epub 2016 Dec 1.


Sickle cell disease: Status with particular reference to India.

Rees DC, Brousse VA.

Indian J Med Res. 2016 Jun;143(6):675-677. doi: 10.4103/0971-5916.191916. No abstract available.


Fragment-to-Lead Medicinal Chemistry Publications in 2015.

Johnson CN, Erlanson DA, Murray CW, Rees DC.

J Med Chem. 2017 Jan 12;60(1):89-99. doi: 10.1021/acs.jmedchem.6b01123. Epub 2016 Oct 14. Review.


Nocturnal enuresis and K+ transport in red blood cells from patients with sickle cell anemia.

Tewari S, Rees DC, Hannemann A, Gbotosho OT, Al Balushi HW, Gibson JS.

Haematologica. 2016 Dec;101(12):e469-e472. Epub 2016 Sep 1. No abstract available.


How benign is sickle cell trait?

Gibson JS, Rees DC.

EBioMedicine. 2016 Sep;11:21-22. doi: 10.1016/j.ebiom.2016.08.023. Epub 2016 Aug 21. No abstract available.


Extracranial internal carotid artery stenosis in children with sickle cell disease - Which transducer, what measurement?

Deane CR, Freedman BJ, Height SE, Rees DC.

Ultrasound. 2016 May;24(2):86-93. doi: 10.1177/1742271X16638891. Epub 2016 Mar 16.


Prasugrel for Sickle Cell Vaso-Occlusive Events.

Heeney MM, Hoppe CC, Rees DC.

N Engl J Med. 2016 Jul 14;375(2):185-6. doi: 10.1056/NEJMc1603499. No abstract available.


Haemoglobinopathies and the rheumatologist.

Hughes M, Akram Q, Rees DC, Jones AK.

Rheumatology (Oxford). 2016 Dec;55(12):2109-2118. Epub 2016 Mar 27. Review.


Nitrogenase FeMoco investigated by spatially resolved anomalous dispersion refinement.

Spatzal T, Schlesier J, Burger EM, Sippel D, Zhang L, Andrade SL, Rees DC, Einsle O.

Nat Commun. 2016 Mar 14;7:10902. doi: 10.1038/ncomms10902.


Cation Homeostasis in Red Cells From Patients With Sickle Cell Disease Heterologous for HbS and HbC (HbSC Genotype).

Hannemann A, Rees DC, Tewari S, Gibson JS.

EBioMedicine. 2015 Sep 18;2(11):1669-76. doi: 10.1016/j.ebiom.2015.09.026. eCollection 2015 Nov.

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