T H J Goodship

Summary

Affiliation: University of Newcastle
Country: UK

Publications

  1. ncbi request reprint 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
    R Thomas Jagoe
    Department of Medicine, University of Newcastle upon Tyne, Newcastle upon Tyne NE2 4HH, UK
    Clin Sci (Lond) 102:353-61. 2002
  2. ncbi request reprint Disordered calcium crystal handling in antisense CLC-5-treated collecting duct cells
    John A Sayer
    Department of Medicine, University of Newcastle upon Tyne, Medical School, Framlington Place, Newcastle upon Tyne, NE2 4HH, UK
    Biochem Biophys Res Commun 300:305-10. 2003
  3. ncbi request reprint Atypical HUS and complement dysregulation
    Timothy H J Goodship
    J Am Soc Nephrol 17:1775-6. 2006
  4. ncbi request reprint Factor H genotype-phenotype correlations: lessons from aHUS, MPGN II, and AMD
    T H J Goodship
    Institute of Human Genetics, University of Newcastle upon Tyne, Newcastle upon Tyne, UK
    Kidney Int 70:12-3. 2006
  5. doi request reprint Factor H autoantibodies in membranoproliferative glomerulonephritis
    Timothy H J Goodship
    Institutes of Cellular Medicine and Genetic Medicine, Newcastle University, Newcastle upon Tyne, UK
    Mol Immunol 52:200-6. 2012
  6. ncbi request reprint Mutations in CD46, a complement regulatory protein, predispose to atypical HUS
    Timothy H J Goodship
    Institute of Human Genetics, University of Newcastle upon Tyne, NE 13BZ, UK
    Trends Mol Med 10:226-31. 2004
  7. ncbi request reprint Long-term follow-up of patients presenting to adult nephrologists with chronic pyelonephritis and 'normal' renal function
    T H Goodship
    Department of Nephrology, School of Clinical Medical Sciences, and Department of Radiology, University of Newcastle upon Tyne
    QJM 93:799-803. 2000
  8. pmc Association of factor H autoantibodies with deletions of CFHR1, CFHR3, CFHR4, and with mutations in CFH, CFI, CD46, and C3 in patients with atypical hemolytic uremic syndrome
    Iain Moore
    Institute of Human Genetics, Newcastle University, Newcastle upon Tyne, United Kingdom
    Blood 115:379-87. 2010
  9. ncbi request reprint Does complement factor B have a role in the pathogenesis of atypical HUS?
    David Kavanagh
    Washington University School of Medicine, Campus Box 8045, St Louis, MO 63110, USA
    Mol Immunol 43:856-9. 2006
  10. doi request reprint Atypical hemolytic uremic syndrome
    David Kavanagh
    The Institute of Human Genetics, International Centre for Life, Newcastle University, Newcastle upon Tyne, UK
    Curr Opin Hematol 17:432-8. 2010

Collaborators

Detail Information

Publications39

  1. ncbi request reprint 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
    R Thomas Jagoe
    Department of Medicine, University of Newcastle upon Tyne, Newcastle upon Tyne NE2 4HH, UK
    Clin Sci (Lond) 102:353-61. 2002
    ....
  2. ncbi request reprint Disordered calcium crystal handling in antisense CLC-5-treated collecting duct cells
    John A Sayer
    Department of Medicine, University of Newcastle upon Tyne, Medical School, Framlington Place, Newcastle upon Tyne, NE2 4HH, UK
    Biochem Biophys Res Commun 300:305-10. 2003
    ..We conclude that expression of CLC-5 in mIMCD-3 cells allows acidification of endosomes and endocytosis, and that disruption of CLC-5 expression causes abnormal crystal agglomeration...
  3. ncbi request reprint Atypical HUS and complement dysregulation
    Timothy H J Goodship
    J Am Soc Nephrol 17:1775-6. 2006
  4. ncbi request reprint Factor H genotype-phenotype correlations: lessons from aHUS, MPGN II, and AMD
    T H J Goodship
    Institute of Human Genetics, University of Newcastle upon Tyne, Newcastle upon Tyne, UK
    Kidney Int 70:12-3. 2006
    ..The report of Licht et al. of a mutation in the complement-regulatory N-terminal region of Factor H in MPGN II provides additional insight into the pathogenesis of this condition...
  5. doi request reprint Factor H autoantibodies in membranoproliferative glomerulonephritis
    Timothy H J Goodship
    Institutes of Cellular Medicine and Genetic Medicine, Newcastle University, Newcastle upon Tyne, UK
    Mol Immunol 52:200-6. 2012
    ..Antibody depleting therapy may have a role in such patients and we suggest that screening for factor H autoantibodies should be undertaken in all patients with MPGN...
  6. ncbi request reprint Mutations in CD46, a complement regulatory protein, predispose to atypical HUS
    Timothy H J Goodship
    Institute of Human Genetics, University of Newcastle upon Tyne, NE 13BZ, UK
    Trends Mol Med 10:226-31. 2004
    ..In the other two, a substitution (S206P) resulted in cell-surface expression but inefficient inactivation of surface-bound C3b. These findings provide further evidence that complement dysregulation predisposes to the development of HUS...
  7. ncbi request reprint Long-term follow-up of patients presenting to adult nephrologists with chronic pyelonephritis and 'normal' renal function
    T H Goodship
    Department of Nephrology, School of Clinical Medical Sciences, and Department of Radiology, University of Newcastle upon Tyne
    QJM 93:799-803. 2000
    ..Most could be referred back to their general practitioner for long-term follow-up...
  8. pmc Association of factor H autoantibodies with deletions of CFHR1, CFHR3, CFHR4, and with mutations in CFH, CFI, CD46, and C3 in patients with atypical hemolytic uremic syndrome
    Iain Moore
    Institute of Human Genetics, Newcastle University, Newcastle upon Tyne, United Kingdom
    Blood 115:379-87. 2010
    ..In 5 patients mutations were identified: 1 in CFH, 1 in CFI, 1 in CD46, and 2 in C3. The latter observation emphasizes that multiple concurrent factors may be necessary in individual patients for disease manifestation...
  9. ncbi request reprint Does complement factor B have a role in the pathogenesis of atypical HUS?
    David Kavanagh
    Washington University School of Medicine, Campus Box 8045, St Louis, MO 63110, USA
    Mol Immunol 43:856-9. 2006
    ..In conclusion, in this small series of aHUS patients we found no evidence that fB has a major role in the pathogenesis of aHUS...
  10. doi request reprint Atypical hemolytic uremic syndrome
    David Kavanagh
    The Institute of Human Genetics, International Centre for Life, Newcastle University, Newcastle upon Tyne, UK
    Curr Opin Hematol 17:432-8. 2010
    ..Many different predisposing genetic factors resulting in complement overactivation have been described in aHUS. Additionally, autoantibodies against complement regulatory proteins have been reported...
  11. pmc Mutations in human complement regulator, membrane cofactor protein (CD46), predispose to development of familial hemolytic uremic syndrome
    Anna Richards
    Institute of Human Genetics, University of Newcastle upon Tyne, Newcastle NE1 4LP, United Kingdom
    Proc Natl Acad Sci U S A 100:12966-71. 2003
    ..This study presents further evidence that complement dysregulation predisposes to development of thrombotic microangiopathy and that screening patients for such defects could provide informed treatment strategies...
  12. doi request reprint Complement polymorphisms: geographical distribution and relevance to disease
    L Ermini
    Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, UK
    Immunobiology 217:265-71. 2012
    ..The analysis of genetic variation in complement genes is a promising tool to unravel mechanisms of host-pathogen interaction and can provide new insights into the evolution of the human immune system...
  13. doi request reprint Is complement factor H a susceptibility factor for IgA nephropathy?
    Matthew Edey
    Institute of Human Genetics, Newcastle University, Newcastle upon Tyne, UK
    Mol Immunol 46:1405-8. 2009
    ..All the patients had a normal serum factor H concentration. In this small cohort of IgA nephropathy patients we have not found evidence to support the hypothesis that factor H is a major susceptibility factor for the disease...
  14. pmc Atypical haemolytic uraemic syndrome associated with a hybrid complement gene
    Julian P Venables
    Institute of Human Genetics, University of Newcastle upon Tyne, Newcastle upon Tyne, United Kingdom
    PLoS Med 3:e431. 2006
    ..In this study we tested the hypothesis that nonallelic homologous recombination between low-copy repeats in the RCA cluster could result in the formation of a hybrid CFH/CFHL1 gene that predisposes to the development of aHUS...
  15. ncbi request reprint Mutations in complement factor I predispose to development of atypical hemolytic uremic syndrome
    David Kavanagh
    Institute of Human Genetics, University of Newcastle upon Tyne, Tyne and Wear NE1 3BZ, UK
    J Am Soc Nephrol 16:2150-5. 2005
    ..As with CFH- and MCP-associated HUS, there was incomplete penetrance in the family of one of the affected individuals. This study provides further evidence that atypical HUS is a disease of complement dysregulation...
  16. doi request reprint Association of a factor H mutation with hemolytic uremic syndrome following a diarrheal illness
    Matthew M Edey
    Department of Nephrology, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK
    Am J Kidney Dis 51:487-90. 2008
    ..We recommend consideration of complement gene mutations in any patient with HUS after a diarrheal episode in which there are unusual features...
  17. ncbi request reprint Membrane cofactor protein and factor I: mutations and transplantation
    David Kavanagh
    Institute of Human Genetics, University of Newcastle upon Tyne, Newcastle upon Tyne, United Kingdom
    Semin Thromb Hemost 32:155-9. 2006
    ..Combined liver/kidney transplantation for patients known to have a CFH mutation has not been successful to date. There is optimism that in the future, targeted complement inhibitors will be of major therapeutic benefit in this condition...
  18. ncbi request reprint The genetics and pathogenesis of haemolytic uraemic syndrome and thrombotic thrombocytopenic purpura
    Anna Richards
    The Institute of Human Genetics and School of Clinical Medical Sciences, University of Newcastle upon Tyne, Newcastle upon Tyne NE1 4LP, UK
    Curr Opin Nephrol Hypertens 11:431-5. 2002
    ..In recent years there has been a substantial increase in the understanding of the genetics and pathogenesis of haemolytic uraemic syndrome and thrombotic thrombocytopenic purpura...
  19. pmc Whole-genome linkage and association scan in primary, nonsyndromic vesicoureteric reflux
    Heather J Cordell
    Institute of Human Genetics, Newcastle University, International Centre for Life, Newcastle upon Tyne, NE1 3BZ, UK
    J Am Soc Nephrol 21:113-23. 2010
    ..The results of this study, which is the largest to date investigating the genetics of reflux, suggest that major loci may not exist for this common renal tract malformation within European populations...
  20. ncbi request reprint Recurrence of hemolytic uremic syndrome after live related renal transplantation associated with subsequent de novo disease in the donor
    Rosemary L Donne
    School of Clinical Medical Sciences, University of Newcastle upon Tyne, England
    Am J Kidney Dis 40:E22. 2002
    ..Neither family has a factor H mutation. This report underlines the risk of disease recurrence in recipients associated with live-related renal transplantation in HUS and also suggests that the donors may be at risk...
  21. pmc Deletion of complement factor H-related genes CFHR1 and CFHR3 is associated with atypical hemolytic uremic syndrome
    Peter F Zipfel
    Leibniz Institute for Natural Product Research and Infection Biology, Hans Knoell Institute, Jena, Germany
    PLoS Genet 3:e41. 2007
    ..The identification of CFHR1/CFHR3 deficiency in aHUS patients may lead to the design of new diagnostic approaches, such as enhanced testing for these genes...
  22. ncbi request reprint A novel non-synonymous polymorphism (p.Arg240His) in C4b-binding protein is associated with atypical hemolytic uremic syndrome and leads to impaired alternative pathway cofactor activity
    Anna M Blom
    Lund University, Department of Laboratory Medicine, Malmo University Hospital, Malmo, Sweden
    J Immunol 180:6385-91. 2008
    ..Three of the patients carry also mutations in membrane cofactor protein and factor H strengthening the hypothesis that individuals may carry multiple susceptibility factors with an additive effect on the risk of developing aHUS...
  23. pmc Complement factor H and the hemolytic uremic syndrome
    John P Atkinson
    Division of Rheumatology, Washington University School of Medicine, St Louis, MO 63110, USA
    J Exp Med 204:1245-8. 2007
    ..A mouse model of HUS designed to mirror human mutations in FH has now been developed, providing new understanding of the molecular pathogenesis of complement-related endothelial disorders...
  24. pmc Modeling how CD46 deficiency predisposes to atypical hemolytic uremic syndrome
    M Kathryn Liszewski
    Washington University School of Medicine, 660 South Euclid Avenue, Campus Box 8045, St Louis, MO 63110, USA
    Mol Immunol 44:1559-68. 2007
    ..Our results add new information relative to the necessity for appropriate expression levels of MCP and further implicate the alternative pathway in disease processes such as aHUS...
  25. doi request reprint Efficacy of plasma therapy in atypical hemolytic uremic syndrome with complement factor H mutations
    Anne Laure Lapeyraque
    Department of Pediatrics, Division of Nephrology, CHU Sainte Justine, Montreal, QC, Canada
    Pediatr Nephrol 23:1363-6. 2008
    ..Long-term tolerance and efficacy of such intensive plasma therapy are still unknown. Reported secondary failure of plasma therapy in factor H deficiency warrants the search for alternative therapeutic approaches...
  26. ncbi request reprint 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
    Stephen J Perkins
    Department of Biochemistry and Molecular Biology, Royal Free and University College Medical School, University College London, Rowland Hill Street, London, NW3 2PF, UK
    J Mol Biol 316:217-24. 2002
    ..As the remaining FH substitutions could also be correlated with their proximity to conserved basic residues, haemolytic uraemic syndrome may result from a failure of FH to interact with polyanions at cell surfaces in the kidney...
  27. ncbi request reprint An interactive web database of factor H-associated hemolytic uremic syndrome mutations: insights into the structural consequences of disease-associated mutations
    Rebecca E Saunders
    Department of Biochemistry and Molecular Biology, Royal Free and University College Medical School, University College London, London, United Kingdom
    Hum Mutat 27:21-30. 2006
    ..This FH web database will facilitate the interpretation of new mutations and polymorphisms when these are identified in patients, and it will clarify the functional role of FH...
  28. ncbi request reprint Lack of major involvement of human uroplakin genes in vesicoureteral reflux: implications for disease heterogeneity
    Songshan Jiang
    Epithelial Biology Unit, Ronald O Perelman Department of Dermatology, Kaplan Comprehensive Cancer Center, New York University Medical School, New York, New York, USA
    Kidney Int 66:10-9. 2004
    ..Recent studies indicate that genetic ablation of mouse uroplakin (UP) III gene, which encodes a 47 kD urothelial-specific integral membrane protein forming urothelial plaques, causes VUR and hydronephrosis...
  29. pmc Membrane cofactor protein mutations in atypical hemolytic uremic syndrome (aHUS), fatal Stx-HUS, C3 glomerulonephritis, and the HELLP syndrome
    Celia J Fang
    Department of Medicine, Washington University School of Medicine, St Louis, MO, USA
    Blood 111:624-32. 2008
    ..Both the R69W and A304V MCP mutations were deficient in their ability to control the alternative pathway of complement activation on a cell surface, illustrating the importance of modeling transmembrane proteins in situ...
  30. ncbi request reprint Outcome of renal transplantation in patients with non-Shiga toxin-associated hemolytic uremic syndrome: prognostic significance of genetic background
    Elena Bresin
    Mario Negri Institute for Pharmacological Research, Clinical Research Center for Rare Diseases Aldo e Cele Daccò, Via Camozzi, 3, 24020 RANICA Bergamo, Italy
    Clin J Am Soc Nephrol 1:88-99. 2006
    ..It is debatable whether a kidney transplant should be recommended for patients with CFH or IF mutation. Reasonably, patients with an MCP mutation can undergo a kidney transplant without risk for recurrence...
  31. ncbi request reprint Atypical relapse of hemolytic uremic syndrome after transplantation
    Karolien H Olie
    Pediatric Nephrology Unit, Emma Children s Hospital, Academic Medical Center, Amsterdam, The Netherlands
    Pediatr Nephrol 19:1173-6. 2004
    ..This patient demonstrates that HUS can recur in a kidney transplant without the diagnostic hematological features and emphasizes the need for early transplant biopsy in such patients showing a decline in transplant function...
  32. ncbi request reprint Posttransplantation cytomegalovirus-induced recurrence of atypical hemolytic uremic syndrome associated with a factor H mutation: successful treatment with intensive plasma exchanges and ganciclovir
    Karolien H Olie
    Paediatric Nephrology Unit, Emma Children s Hospital Academic Medical Centre, Amsterdam, The Netherlands
    Am J Kidney Dis 45:e12-5. 2005
    ..The feasibility of kidney transplantation in case of atypical HUS related to factor H mutation using continuous prophylactic PE intensified during relapses should be confirmed in prospective studies...
  33. ncbi request reprint Complement factor H-associated atypical hemolytic uremic syndrome in monozygotic twins: concordant presentation, discordant response to treatment
    Jean Claude Davin
    Emma Children s Hospital, Department of Pediatric Nephrology and Pathology, Academic Medical Centre, Amsterdam, The Netherlands
    Am J Kidney Dis 47:e27-30. 2006
    ..73 m2 [1.98 mL/s/1.73 m2]) on maintenance PE therapy. In conclusion, the response to treatment of these monozygotic twins suggests that long-term PE may have benefits over plasma infusion alone...
  34. ncbi request reprint De novo gene conversion in the RCA gene cluster (1q32) causes mutations in complement factor H associated with atypical hemolytic uremic syndrome
    Stefan Heinen
    Department for Infection Biology, Hans Knoell Institute for Natural Products Research, Jena, Germany
    Hum Mutat 27:292-3. 2006
    ..We, therefore, provide conclusive evidence that gene conversion is responsible for functionally significant CFH mutations in aHUS...
  35. ncbi request reprint Implications of the initial mutations in membrane cofactor protein (MCP; CD46) leading to atypical hemolytic uremic syndrome
    Anna Richards
    Department of Medicine, Division of Rheumatology, Washington University School of Medicine, Campus Box 8045, 660 South Euclid Avenue, St Louis, MO 63110, USA
    Mol Immunol 44:111-22. 2007
    ..This highlights the importance of defining and characterizing the underlying genetic defects in patients with aHUS...
  36. ncbi request reprint Atypical haemolytic uraemic syndrome
    David Kavanagh
    Washington University School of Medicine, St Louis, MO 63110, USA
    Br Med Bull 77:5-22. 2006
    ..Discovery of these mutations has revealed important genotype-phenotype correlations. MCP-HUS has a better prognosis and a better outcome after transplantation than either CFH-HUS or IF-HUS...
  37. ncbi request reprint The interactive Factor H-atypical hemolytic uremic syndrome mutation database and website: update and integration of membrane cofactor protein and Factor I mutations with structural models
    Rebecca E Saunders
    Department of Biochemistry and Molecular Biology, Royal Free and University College Medical School, University College London, London, United Kingdom
    Hum Mutat 28:222-34. 2007
    ..The database emphasizes the causative role of the alternative pathway of complement in disease and provides a repository of knowledge to assist future diagnosis and novel therapeutic approaches...
  38. ncbi request reprint Characterization of mutations in complement factor I (CFI) associated with hemolytic uremic syndrome
    David Kavanagh
    Division of Rheumatology, Washington University School of Medicine, St Louis, MO 63110, USA
    Mol Immunol 45:95-105. 2008
    ..The excessive complement activation for a given degree of damage may result in generation of a procoagulant state and aHUS...
  39. ncbi request reprint Solitary functioning kidney and diverse genital tract malformations associated with hepatocyte nuclear factor-1beta mutations
    Coralie Bingham
    Department of Vascular Medicine and Diabetes Research, School of Postgraduate Medicine and Health Sciences, University of Exeter, Devon, United Kingdom
    Kidney Int 61:1243-51. 2002
    ..Renal and uterine abnormalities have not been described in families without early-onset diabetes...