Timothy Goodship

Timothy Goodship

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Timothy Goodship

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Clinical Relapses of Atypical HUS on Eculizumab: Clinical Gap for Monitoring and Individualised Therapy.

Case Rep Nephrol 2018 6;2018:2781789. Epub 2018 Feb 6.

Department of Paediatric Nephrology & Transplantation, The Children's University Hospital, Temple Street, Dublin 1, Ireland.

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http://dx.doi.org/10.1155/2018/2781789DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5818949PMC
February 2018

Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a "Kidney Disease: Improving Global Outcomes" (KDIGO) Controversies Conference.

Kidney Int 2017 03 16;91(3):539-551. Epub 2016 Dec 16.

Molecular Otolaryngology and Renal Research Laboratories, Carver College of Medicine, University of Iowa, Iowa City, Iowa, USA; Division of Nephrology, Department of Internal Medicine, Carver College of Medicine, University of Iowa, Iowa City, Iowa, USA. Electronic address:

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http://dx.doi.org/10.1016/j.kint.2016.10.005DOI Listing
March 2017

Chromosomal rearrangement-A rare cause of complement factor I associated atypical haemolytic uraemic syndrome.

Immunobiology 2016 10 10;221(10):1124-30. Epub 2016 May 10.

Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, UK. Electronic address:

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http://dx.doi.org/10.1016/j.imbio.2016.05.002DOI Listing
October 2016

Patient stratification and therapy in atypical haemolytic uraemic syndrome (aHUS).

Immunobiology 2016 06 11;221(6):715-8. Epub 2015 May 11.

Institute of Genetic Medicine and Cellular Medicine, Newcastle University, United Kingdom. Electronic address:

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http://dx.doi.org/10.1016/j.imbio.2015.05.002DOI Listing
June 2016

Use of eculizumab in crescentic IgA nephropathy: proof of principle and conundrum?

Clin Kidney J 2015 Oct 27;8(5):489-91. Epub 2015 Aug 27.

Institute of Genetic Medicine , Newcastle University , Newcastle upon Tyne , UK.

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http://dx.doi.org/10.1093/ckj/sfv076DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4581393PMC
October 2015

Case report: Benefits and challenges of long-term eculizumab in atypical hemolytic uremic syndrome.

Pediatrics 2015 Jun 4;135(6):e1506-9. Epub 2015 May 4.

The Department for Paediatric Nephrology & Transplantation, The Children's University Hospital, Dublin 1, Ireland; and.

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http://dx.doi.org/10.1542/peds.2014-3503DOI Listing
June 2015

Factors determining penetrance in familial atypical haemolytic uraemic syndrome.

J Med Genet 2014 Nov 26;51(11):756-64. Epub 2014 Sep 26.

Peninsula Clinical Genetics Service, Royal Devon & Exeter NHS Foundation Trust, Royal Devon & Exeter Hospital (Heavitree), Exeter, UK University of Exeter Medical School, University of Exeter, Exeter, UK.

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http://dx.doi.org/10.1136/jmedgenet-2014-102498DOI Listing
November 2014

Atypical haemolytic uraemic syndrome associated with a mutation triggered by

Clin Kidney J 2014 Jun 11;7(3):286-288. Epub 2014 Apr 11.

Renal Services Centre , Freeman Hospital , Newcastle upon Tyne , UK ; The Institute of Genetic Medicine , Newcastle University , Newcastle upon Tyne , UK.

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http://dx.doi.org/10.1093/ckj/sfu032DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4038258PMC
June 2014

Genotype/phenotype correlations in complement factor H deficiency arising from uniparental isodisomy.

Am J Kidney Dis 2013 Nov 16;62(5):978-83. Epub 2013 Jul 16.

Northern Molecular Genetics Service, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, United Kingdom.

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http://dx.doi.org/10.1053/j.ajkd.2013.05.020DOI Listing
November 2013

Prevalence in the General Population of a CFH Sequence Variant Associated with Atypical Haemolytic Uraemic Syndrome in an Extensive Family from Southwest England.

Nephron Extra 2013 17;3(1):86-90. Epub 2013 Sep 17.

NIHR Exeter Clinical Research Facility, University of Exeter Medical School, Plymouth, UK ; Renal Unit, Royal Devon and Exeter Hospital, Exeter, Plymouth, UK.

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http://dx.doi.org/10.1159/000354667DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3806708PMC
October 2013

Eculizumab therapy for atypical haemolytic uraemic syndrome due to a gain-of-function mutation of complement factor B.

Pediatr Nephrol 2013 Aug 28;28(8):1315-8. Epub 2013 Apr 28.

Regional Paediatric Nephro-Urology Unit, Southampton Children's Hospital, Tremona Road, Southampton, SO16 6YD, UK.

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http://dx.doi.org/10.1007/s00467-013-2492-xDOI Listing
August 2013

Cisplatin-induced haemolytic uraemic syndrome associated with a novel intronic mutation of treated with eculizumab.

Clin Kidney J 2013 Aug;6(4):421-425

Institute of Genetic Medicine , Newcastle University, International Centre for Life , Newcastle upon Tyne , UK.

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http://dx.doi.org/10.1093/ckj/sft065DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3888095PMC
August 2013

A complementary component to atypical haemolytic uraemic syndrome.

BMJ Case Rep 2013 Jun 18;2013. Epub 2013 Jun 18.

Department of Nephrology, Royal Free Hospital NHS Trust, London, UK.

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http://dx.doi.org/10.1136/bcr-2013-010214DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3702985PMC
June 2013

Combined complement gene mutations in atypical hemolytic uremic syndrome influence clinical phenotype.

J Am Soc Nephrol 2013 Feb 21;24(3):475-86. Epub 2013 Feb 21.

Clinical Research Center for Rare Diseases, “Aldo e Cele Daccò,” Mario Negri Institute for Pharmacological Research, Ranica, Bergamo, Italy.

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http://dx.doi.org/10.1681/ASN.2012090884DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3582207PMC
February 2013

Atypical hemolytic uremic syndrome, genetic basis, and clinical manifestations.

Hematology Am Soc Hematol Educ Program 2011 ;2011:15-20

Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, United Kingdom.

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http://dx.doi.org/10.1182/asheducation-2011.1.15DOI Listing
April 2012

A novel hybrid CFH/CFHR3 gene generated by a microhomology-mediated deletion in familial atypical hemolytic uremic syndrome.

Blood 2012 Jan 4;119(2):591-601. Epub 2011 Nov 4.

Department of Infection, Immunity & Biochemistry, Cardiff University School of Medicine, Heath Park, United Kingdom.

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http://dx.doi.org/10.1182/blood-2011-03-339903DOI Listing
January 2012

Non-atheromatous arterial stenoses in atypical haemolytic uraemic syndrome associated with complement dysregulation.

Nephrol Dial Transplant 2010 Oct 8;25(10):3421-5. Epub 2010 Jun 8.

Pediatric Nephrology Department, Hôpital Robert Debré, Assistance Publique-Hôpitaux de Paris, Université Paris 7, Paris, France.

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https://academic.oup.com/ndt/article-lookup/doi/10.1093/ndt/
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http://dx.doi.org/10.1093/ndt/gfq319DOI Listing
October 2010

Atypical hemolytic uremic syndrome.

Curr Opin Hematol 2010 Sep;17(5):432-8

The Institute of Human Genetics, International Centre for Life, Newcastle University, Newcastle upon Tyne, UK.

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http://dx.doi.org/10.1097/MOH.0b013e32833cae86DOI Listing
September 2010

Triggering of atypical hemolytic uremic syndrome by influenza A (H1N1).

Ren Fail 2010 Jul;32(6):753-6

Intensive Care Unit, Hospital de Santa Cruz, Centro Hospitalar de Lisboa Ocidental, Lisbon, Portugal.

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http://www.tandfonline.com/doi/full/10.3109/0886022X.2010.48
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http://dx.doi.org/10.3109/0886022X.2010.486491DOI Listing
July 2010

Pulling the trigger in atypical hemolytic uremic syndrome: the role of pregnancy.

J Am Soc Nephrol 2010 May 15;21(5):731-2. Epub 2010 Apr 15.

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http://dx.doi.org/10.1681/ASN.2010030308DOI Listing
May 2010

Is complement factor H a susceptibility factor for IgA nephropathy?

Mol Immunol 2009 Apr 21;46(7):1405-8. Epub 2009 Jan 21.

Institute of Human Genetics, Newcastle University, Newcastle upon Tyne, UK.

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http://dx.doi.org/10.1016/j.molimm.2008.12.002DOI Listing
April 2009

Efficacy of plasma therapy in atypical hemolytic uremic syndrome with complement factor H mutations.

Pediatr Nephrol 2008 Aug 19;23(8):1363-6. Epub 2008 Apr 19.

Department of Pediatrics, Division of Nephrology, CHU Sainte-Justine, Montreal, QC, Canada.

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http://dx.doi.org/10.1007/s00467-008-0803-4DOI Listing
August 2008

Association of a factor H mutation with hemolytic uremic syndrome following a diarrheal illness.

Am J Kidney Dis 2008 Mar;51(3):487-90

Department of Nephrology, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK.

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http://dx.doi.org/10.1053/j.ajkd.2007.08.030DOI Listing
March 2008

Characterization of mutations in complement factor I (CFI) associated with hemolytic uremic syndrome.

Mol Immunol 2008 Jan 26;45(1):95-105. Epub 2007 Jun 26.

Division of Rheumatology, Washington University School of Medicine, St. Louis, MO 63110, USA.

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http://dx.doi.org/10.1016/j.molimm.2007.05.004DOI Listing
January 2008

Update on evaluating complement in hemolytic uremic syndrome.

Curr Opin Nephrol Hypertens 2007 Nov;16(6):565-71

Renal Division, Institute of Human Genetics, University of Newcastle upon Tyne, Newcastle upon Tyne, United Kingdom.

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http://dx.doi.org/10.1097/MNH.0b013e3282f0872fDOI Listing
November 2007

Where next with atypical hemolytic uremic syndrome?

Mol Immunol 2007 Sep;44(16):3889-900

Department of Bacteriology and Immunology, Haartman Institute and HUSLAB, University of Helsinki and Helsinki University Central Hospital, FIN-00290 Helsinki, Finland.

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http://dx.doi.org/10.1016/j.molimm.2007.06.003DOI Listing
September 2007

Complement factor H and the hemolytic uremic syndrome.

J Exp Med 2007 Jun 4;204(6):1245-8. Epub 2007 Jun 4.

Division of Rheumatology, Washington University School of Medicine, St. Louis, MO 63110, USA.

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http://dx.doi.org/10.1084/jem.20070664DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2118604PMC
June 2007

Screening for complement system abnormalities in patients with atypical hemolytic uremic syndrome.

Clin J Am Soc Nephrol 2007 May 14;2(3):591-6. Epub 2007 Feb 14.

Division of Rheumatology, Washington University School of Medicine, St. Louis, Missouri 63110, USA.

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http://dx.doi.org/10.2215/CJN.03270906DOI Listing
May 2007

Atypical haemolytic uraemic syndrome.

Br Med Bull 2006 11;77-78:5-22. Epub 2006 Sep 11.

Washington University School of Medicine, St Louis, MO 63110, USA.

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http://dx.doi.org/10.1093/bmb/ldl004DOI Listing
March 2007

Modeling how CD46 deficiency predisposes to atypical hemolytic uremic syndrome.

Mol Immunol 2007 Mar 5;44(7):1559-68. Epub 2006 Oct 5.

Washington University School of Medicine, 660 South Euclid Avenue, Campus Box 8045, St. Louis, MO 63110, USA.

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http://dx.doi.org/10.1016/j.molimm.2006.08.024DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1828070PMC
March 2007

Nutritional screening in patients on hemodialysis: is subjective global assessment an appropriate tool?

J Ren Nutr 2007 Mar;17(2):114-7

School of Clinical Medical Sciences, University of Newcastle upon Tyne, UK.

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http://dx.doi.org/10.1053/j.jrn.2006.12.008DOI Listing
March 2007

Anguish over angiopathy: hemolytic uremic syndrome.

Clin Immunol 2007 Feb 14;122(2):135-8. Epub 2006 Sep 14.

Clinical Sciences Lund, Department of Pediatrics, Lund University, Lund, Sweden.

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http://dx.doi.org/10.1016/j.clim.2006.07.010DOI Listing
February 2007

Implications of the initial mutations in membrane cofactor protein (MCP; CD46) leading to atypical hemolytic uremic syndrome.

Mol Immunol 2007 Jan 1;44(1-3):111-22. Epub 2006 Aug 1.

Department of Medicine, Division of Rheumatology, Washington University School of Medicine, Campus Box 8045, 660 South Euclid Avenue, St. Louis, MO 63110, USA.

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http://dx.doi.org/10.1016/j.molimm.2006.07.004DOI Listing
January 2007

A common CFH haplotype, with deletion of CFHR1 and CFHR3, is associated with lower risk of age-related macular degeneration.

Nat Genet 2006 Oct 24;38(10):1173-7. Epub 2006 Sep 24.

Department of Medical Genetics, Queen's University, Belfast, Belfast BT12 6BL, UK.

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http://www.nature.com/articles/ng1890
Publisher Site
http://dx.doi.org/10.1038/ng1890DOI Listing
October 2006

Atypical HUS and complement dysregulation.

J Am Soc Nephrol 2006 Jul 21;17(7):1775-6. Epub 2006 Jun 21.

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http://dx.doi.org/10.1681/ASN.2006050521DOI Listing
July 2006

Telomere shortening and haemodialysis.

Blood Purif 2006 22;24(2):185-9. Epub 2005 Dec 22.

Institute of Human Genetics and Institute for Ageing and Health, University of Newcastle upon Tyne, and Renal Unit, Newcastle upon Tyne Hospitals NHS Trust, Newcastle upon Tyne, UK.

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http://dx.doi.org/10.1159/000090517DOI Listing
June 2006

Does complement factor B have a role in the pathogenesis of atypical HUS?

Mol Immunol 2006 Mar 2;43(7):856-9. Epub 2005 Aug 2.

Washington University School of Medicine, Campus Box 8045, St. Louis, MO 63110, USA.

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http://dx.doi.org/10.1016/j.molimm.2005.06.041DOI Listing
March 2006

Membrane cofactor protein and factor I: mutations and transplantation.

Semin Thromb Hemost 2006 Mar;32(2):155-9

Institute of Human Genetics, University of Newcastle upon Tyne, Newcastle upon Tyne, United Kingdom.

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http://dx.doi.org/10.1055/s-2006-939771DOI Listing
March 2006

Complement factor H-associated atypical hemolytic uremic syndrome in monozygotic twins: concordant presentation, discordant response to treatment.

Am J Kidney Dis 2006 Feb;47(2):e27-30

Emma Children's Hospital, Department of Pediatric Nephrology and Pathology, Academic Medical Centre, Amsterdam, The Netherlands.

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http://dx.doi.org/10.1053/j.ajkd.2005.11.007DOI Listing
February 2006

An interactive web database of factor H-associated hemolytic uremic syndrome mutations: insights into the structural consequences of disease-associated mutations.

Hum Mutat 2006 Jan;27(1):21-30

Department of Biochemistry and Molecular Biology, Royal Free and University College Medical School, University College London, London, United Kingdom.

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http://www.atypicalhus.50megs.com/Saunders20062.pdf
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http://doi.wiley.com/10.1002/humu.20268
Publisher Site
http://dx.doi.org/10.1002/humu.20268DOI Listing
January 2006

Outcome of renal transplantation in patients with non-Shiga toxin-associated hemolytic uremic syndrome: prognostic significance of genetic background.

Clin J Am Soc Nephrol 2006 Jan 2;1(1):88-99. Epub 2005 Nov 2.

Mario Negri Institute for Pharmacological Research, Clinical Research Center for Rare Diseases Aldo e Cele Daccò, Via Camozzi, 3, 24020 RANICA (Bergamo), Italy.

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http://dx.doi.org/10.2215/CJN.00050505DOI Listing
January 2006

Mutations in complement factor I predispose to development of atypical hemolytic uremic syndrome.

J Am Soc Nephrol 2005 Jul 25;16(7):2150-5. Epub 2005 May 25.

Institute of Human Genetics, University of Newcastle upon Tyne, Tyne and Wear NE1 3BZ, UK.

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http://dx.doi.org/10.1681/ASN.2005010103DOI Listing
July 2005

Atypical relapse of hemolytic uremic syndrome after transplantation.

Pediatr Nephrol 2004 Oct 5;19(10):1173-6. Epub 2004 Aug 5.

Pediatric Nephrology Unit, Emma Children's Hospital, Academic Medical Center, Amsterdam, The Netherlands.

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http://dx.doi.org/10.1007/s00467-004-1565-2DOI Listing
October 2004

Challenges in the management of infantile factor H associated hemolytic uremic syndrome.

Pediatr Nephrol 2004 Aug 16;19(8):908-11. Epub 2004 Jun 16.

Department of Pediatrics, Children's Hospital of Eastern Ontario, The Ottawa Hospital, University of Ottawa, 401 Smyth Road, K1H 8L1, Ottawa, Ontario, Canada.

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http://dx.doi.org/10.1007/s00467-004-1526-9DOI Listing
August 2004

Mutations in CD46, a complement regulatory protein, predispose to atypical HUS.

Trends Mol Med 2004 May;10(5):226-31

Institute of Human Genetics, University of Newcastle upon Tyne, NE 13BZ, UK.

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http://dx.doi.org/10.1016/j.molmed.2004.03.006DOI Listing
May 2004

Fibrinogen in hemodialysis: the worst of both worlds?

Kidney Int 2003 Jan;63(1):379-80

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http://dx.doi.org/10.1046/j.1523-1755.2003.00748.xDOI Listing
January 2003

Disordered calcium crystal handling in antisense CLC-5-treated collecting duct cells.

Biochem Biophys Res Commun 2003 Jan;300(2):305-10

Department of Medicine, University of Newcastle Upon Tyne, Medical School, Framlington Place, Newcastle upon Tyne, NE2 4HH, UK.

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http://dx.doi.org/10.1016/s0006-291x(02)02837-1DOI Listing
January 2003

Effects of feeding on albumin synthesis in hypoalbuminemic hemodialysis patients.

Kidney Int 2002 Jul;62(1):266-71

School of Clinical Medical Sciences and Biomedical Mass Spectrometry Unit, Royal Victoria Infirmary, University of Newcastle upon Tyne, Newcastle-upon-Tyne NE1 4LP, England, UK.

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http://dx.doi.org/10.1046/j.1523-1755.2002.00413.xDOI Listing
July 2002

The genetics and pathogenesis of haemolytic uraemic syndrome and thrombotic thrombocytopenic purpura.

Curr Opin Nephrol Hypertens 2002 Jul;11(4):431-5

The Institute of Human Genetics and School of Clinical Medical Sciences, University of Newcastle upon Tyne, Newcastle upon Tyne NE1 4LP, UK.

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http://dx.doi.org/10.1097/00041552-200207000-00010DOI Listing
July 2002

Solitary functioning kidney and diverse genital tract malformations associated with hepatocyte nuclear factor-1beta mutations.

Kidney Int 2002 Apr;61(4):1243-51

Department of Vascular Medicine and Diabetes Research, School of Postgraduate Medicine and Health Sciences, University of Exeter, Devon, United Kingdom.

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http://dx.doi.org/10.1046/j.1523-1755.2002.00272.xDOI Listing
April 2002

Skeletal muscle mRNA levels for cathepsin B, but not components of the ubiquitin-proteasome pathway, are increased in patients with lung cancer referred for thoracotomy.

Clin Sci (Lond) 2002 Mar;102(3):353-61

Department of Medicine, University of Newcastle upon Tyne, Newcastle upon Tyne NE2 4HH, UK.

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March 2002

Molecular modelling of the C-terminal domains of factor H of human complement: a correlation between haemolytic uraemic syndrome and a predicted heparin binding site.

J Mol Biol 2002 Feb;316(2):217-24

Department of Biochemistry and Molecular Biology, Royal Free and University College Medical School, University College London, Rowland Hill Street, London, NW3 2PF, UK.

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http://dx.doi.org/10.1006/jmbi.2001.5337DOI Listing
February 2002