Diagnosis, pathogenesis, and treatment prospects in cystic kidney disease.
Mol Diagn Ther
; 10(3): 163-74, 2006.
Article
en En
| MEDLINE
| ID: mdl-16771602
Cystic kidney diseases (CKDs) are a clinically and genetically heterogeneous group of disorders characterized by progressive fibrocystic renal and hepatobiliary changes. Recent findings have proven the cystogenic process to be compatible with cellular dedifferentiation, i. e. increased apoptosis and proliferation rates, altered protein sorting and secretory characteristics, as well as disorganization of the extracellular matrix. Compelling evidence suggests that cilia play a central pathogenic role and most cystic kidney disorders converge into a common pathogenic pathway. Recently, several promising trials have further extended our understanding of the pathophysiology of CKD and may have the potential for rational personalized therapies in future years. This review aims to summarize the current state of knowledge of the structure and function of proteins underlying polycystic kidney disease, to explore the clinical consequences of changes in respective genes, and to discuss potential therapeutic approaches.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Enfermedades Renales Quísticas
Tipo de estudio:
Diagnostic_studies
/
Etiology_studies
Límite:
Humans
Idioma:
En
Revista:
Mol Diagn Ther
Asunto de la revista:
BIOLOGIA MOLECULAR
/
FARMACOLOGIA
/
TECNICAS E PROCEDIMENTOS DE LABORATORIO
Año:
2006
Tipo del documento:
Article
País de afiliación:
Alemania
Pais de publicación:
Nueva Zelanda