Pathogenesis and New Treatment of Autosomal Dominant Polycystic Kidney Disease / 대한신장학회지
Korean Journal of Nephrology
;
: 231-238, 2011.
Article
in Korean
| WPRIM
| ID: wpr-167523
ABSTRACT
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Biology
/
Vasopressins
/
Proteins
/
Cilia
/
Polycystic Kidney, Autosomal Dominant
/
Kidney
/
Polycystic Kidney Diseases
Type of study:
Etiology study
Language:
Korean
Journal:
Korean Journal of Nephrology
Year:
2011
Type:
Article
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