Sirtuin 1 inhibition delays cyst formation in autosomal-dominant polycystic kidney disease

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Sirtuin 1 inhibition delays cyst formation in autosomal-dominant polycystic kidney disease.

Autosomal-dominant polycystic kidney disease (ADPKD) is caused by mutations in either PKD1 or PKD2 and is characterized by the development of multiple bilateral renal cysts that replace normal kidney tissue. Here, we used Pkd1 mutant mouse models to demonstrate that the nicotinamide adenine dinucleotide-dependent (NAD-dependent) protein deacetylase sirtuin 1 (SIRT1) is involved in the pathophys...

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Surgical cyst decortication in autosomal dominant polycystic kidney disease.

PURPOSE To provide a summary of the relevant literature regarding the impact of surgical cyst decortication on hypertension, renal function, and pain management in patients with autosomal dominant polycystic kidney disease (ADPKD). METHODS Data collection was conducted via a Medline search using the subject headings autosomal dominant polycystic kidney disease, surgery, decortication, and mar...

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Cyst infections in patients with autosomal dominant polycystic kidney disease.

BACKGROUND AND OBJECTIVES Cyst infection is a complex diagnostic and therapeutic issue in patients with autosomal dominant polycystic kidney disease (ADPKD); however, published data regarding the diagnosis and the management of cyst infections in patients with ADPKD are sparse. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS A retrospective study was conducted in a referral center for patients ...

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Autosomal dominant polycystic kidney disease.

The lack of reliable data on frequency, age of onset, survival, spontaneous mutation rate and prognosis in autosomal dominant polycystic kidney disease is a continual source of frustration to physicians involved in counselling patients and their relatives. The only major study to address all of these issues in a defined population was presented by Dalgaard as a 251-page doctoral thesis in 1957 ...

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Autosomal Dominant Polycystic Kidney Disease

recessive forms (1–3), autosomal dominant polycystic kidney disease (ADPKD) with an incidence of 1 : 500 to 1 : 1000 is one of the commonest hereditary diseases (4). Some 5 million people worldwide are affected. In many countries ADPKD is the fourth most frequent cause of end-stage renal failure. About 85% of these diseases are caused by mutations in the PKD1 gene, the remaining 15% are due to ...

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ژورنال

عنوان ژورنال: Journal of Clinical Investigation

سال: 2013

ISSN: 0021-9738

DOI: 10.1172/jci64401