지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
커뮤니티
연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2011.1
- 수록면
- 231 - 238 (8page)
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초록· 키워드
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The discovery of the genes and their respective proteins that are associated with autosomal dominant polycystic kidney disease (ADPKD) has revolutionized the field of ADPKD biology. Recent studies indicate that the pathogenesis of ADPKD is linked to abnormalities in the primary cilium in the kidney. Inactivation of ciliary proteins in the postnatal kidney has uncovered novel roles of primary cilia in regulating tubular growth and repair after injury. Furthermore, defective tubular repair after injury may contribute to the progression of ADPKD. Studies of signaling pathways that are perturbed in ADPKD have identified potential targets for pharmacological therapy. Better understanding of the downstream consequences of ADPKD mutations has identified a number of therapeutic targets that are now being tested in preclinical and clinical trials. The author summarized recent insights in the pathogenesis of ADPKD including the genetics of ADPKD, the properties of the respective polycystin proteins, the role of cilia, some cell-signaling pathways and new therapeutic interventions.
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참고문헌 (59)
참고문헌 신청
Gabow PA / 1993 / Autosomal dominant polycystic kidney disease / N Engl J Med 329:332~342
Yoder BK / 2002 / The polycystic kidney disease proteins, polycystin-1, polycystin-2, polaris, and cystin, are co-localized in renal cilia / J Am Soc Nephrol 13:2508~2516
Rossetti S / 2007 / CRISP Consortium: Comprehensive molecular diagnostics in autosomal dominant polycystic kidney disease / J Am Soc Nephrol 18:2143~2160
Rossetti S / 2002 / The position of the polycystic kidney disease 1 (PKD1) gene mutation correlates with the severity of renal disease / J Am Soc Nephrol 13:1230~1237
Arnaout MA / 2001 / Molecular genetics and pathogenesis of autosomal dominant polycystic kidney disease / Annu Rev Med 52:93~123
Yoder BK / 2002 / The polycystic kidney disease proteins, polycystin-1, polycystin-2, polaris, and cystin, are co-localized in renal cilia / J Am Soc Nephrol 13:2508~2516
Rossetti S / 2007 / CRISP Consortium: Comprehensive molecular diagnostics in autosomal dominant polycystic kidney disease / J Am Soc Nephrol 18:2143~2160
Rossetti S / 2002 / The position of the polycystic kidney disease 1 (PKD1) gene mutation correlates with the severity of renal disease / J Am Soc Nephrol 13:1230~1237
Arnaout MA / 2001 / Molecular genetics and pathogenesis of autosomal dominant polycystic kidney disease / Annu Rev Med 52:93~123
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