Na Transport in Autosomal Recessive Polycystic Kidney Disease (ARPKD) Cyst Lining Epithelial Cells
نویسندگان
چکیده
منابع مشابه
[Autosomal recessive polycystic kidney disease (ARPKD)].
Autosomal recessive polycystic kidney disease (ARPKD) is an important hereditary early childhood nephropathy. However, the clinical ARPKD spectrum is much more variable than is generally presumed. Presentation of ARPKD at a later age and survival into adulthood is well known. Diagnostic criteria, clinical course, differential diagnoses, genetics and molecular biology will be discussed along wit...
متن کاملMechanoregulation of intracellular Ca2+ in human autosomal recessive polycystic kidney disease cyst-lining renal epithelial cells.
Mutations of cilia-expressed proteins are associated with an attenuated shear-induced increase in intracellular Ca(2+) concentration ([Ca(2+)](i)) in renal epithelial cell lines derived from murine models of autosomal recessive polycystic kidney disease (ARPKD). We hypothesized that human ARPKD cyst-lining renal epithelial cells also exhibited dysregulated mechanosensation. To test this, condit...
متن کاملMechanoregulation of intracellular Ca in human autosomal recessive polycystic kidney disease cyst-lining renal epithelial cells
Rohatgi R, Battini L, Kim P, Israeli S, Wilson PD, Gusella GL, Satlin LM. Mechanoregulation of intracellular Ca in human autosomal recessive polycystic kidney disease cyst-lining renal epithelial cells. Am J Physiol Renal Physiol 294: F890–F899, 2008. First published February 6, 2008; doi:10.1152/ajprenal.00341.2007.—Mutations of cilia-expressed proteins are associated with an attenuated shear-...
متن کاملAutosomal Recessive Polycystic Kidney Disease
The following fictional case is intended as a learning tool within the Pathology Competencies for Medical Education (PCME), a set of national standards for teaching pathology. These are divided into three basic competencies: Disease Mechanisms and Processes, Organ System Pathology, and Diagnostic Medicine and Therapeutic Pathology. For additional information, and a full list of learning objecti...
متن کاملAutosomal Recessive Polycystic Kidney Disease
A single gene defect leads to differing degrees of renal and hepatic involvement, with very different phenotypes and clinical outcome within even one affected family. [2] Kidneys are bilaterally enlarged and contain large numbers of cysts throughout the organ, due to the dilatation and elongation of renal collecting ducts. At birth, the interstitium and the rest of the tubules are normal but th...
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ژورنال
عنوان ژورنال: Journal of the American Society of Nephrology
سال: 2003
ISSN: 1046-6673,1533-3450
DOI: 10.1097/01.asn.0000056481.66379.b2