نتایج جستجو برای: nephropathic

تعداد نتایج: 729  

Journal: :Clinical Journal of the American Society of Nephrology 2008

2016
Wai W. Cheung Stephanie Cherqui Wei Ding Mary Esparza Ping Zhou Jianhua Shao Richard L. Lieber Robert H. Mak

BACKGROUND Muscle wasting is a common complication in patients with infantile nephropathic cystinosis, but its mechanism and association with energy metabolism is not known. We define the metabolic phenotype in Ctns(-/-) mice, an established murine model of infantile nephropathic cystinosis, with focus on muscle wasting and energy homeostasis. METHODS Male Ctns(-/-) mice and wild-type (WT) co...

Journal: :Clinical journal of the American Society of Nephrology : CJASN 2008
Aude Servais Vincent Morinière Jean-Pierre Grünfeld Laure-Hélène Noël Jean-Michel Goujon Bernadette Chadefaux-Vekemans Corinne Antignac

BACKGROUND AND OBJECTIVES Cystinosis is an autosomal recessive disease characterized by the intralysosomal accumulation of cystine, as a result of a defect in cystine transport across the lysosomal membrane. Three clinical forms have been described on the basis of severity of symptoms and age of onset: infantile cystinosis, characterized by renal proximal tubulopathy and progression to end-stag...

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