Polycystic kidney disease

C Bergmann, LM Guay-Woodford, PC Harris… - Nature reviews Disease …, 2018 - nature.com
Cystic kidneys are common causes of end-stage renal disease, both in children and in
adults. Autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive …

Renal cystic diseases: a review

M Bisceglia, CA Galliani, C Senger… - Advances in anatomic …, 2006 - journals.lww.com
This review aims to assist in the categorization of inherited, developmental, and acquired
cystic disease of the kidney as well as to provide a pertinent, up-to-date bibliography. The …

Organoid-on-a-chip model of human ARPKD reveals mechanosensing pathomechanisms for drug discovery

K Hiratsuka, T Miyoshi, KT Kroll, NR Gupta… - Science …, 2022 - science.org
Organoids serve as a novel tool for disease modeling in three-dimensional multicellular
contexts. Static organoids, however, lack the requisite biophysical microenvironment such …

Increased hepatic CD36 expression contributes to dyslipidemia associated with diet-induced obesity

DPY Koonen, RL Jacobs, M Febbraio, ME Young… - …, 2007 - Am Diabetes Assoc
OBJECTIVE—The etiology of type 2 diabetes often involves diet-induced obesity (DIO),
which is associated with elevated plasma fatty acids and lipoprotein associated triglycerides …

Wnt9b signaling regulates planar cell polarity and kidney tubule morphogenesis

CM Karner, R Chirumamilla, S Aoki, P Igarashi… - Nature …, 2009 - nature.com
Although many vertebrate organs, such as kidneys, lungs and liver, are composed of
epithelial tubules, little is known of the mechanisms that establish the length or diameter of …

[HTML][HTML] Genetics of autosomal recessive polycystic kidney disease and its differential diagnoses

C Bergmann - Frontiers in pediatrics, 2018 - frontiersin.org
Autosomal recessive polycystic kidney disease (ARPKD) is a hepatorenal fibrocystic
disorder that is characterized by enlarged kidneys with progressive loss of renal function …

Pathobiology of inherited biliary diseases: a roadmap to understand acquired liver diseases

L Fabris, R Fiorotto, C Spirli, M Cadamuro… - Nature Reviews …, 2019 - nature.com
Bile duct epithelial cells, also known as cholangiocytes, regulate the composition of bile and
its flow. Acquired, congenital and genetic dysfunctions in these cells give rise to a set of …

Cholangiocyte cilia detect changes in luminal fluid flow and transmit them into intracellular Ca2+ and cAMP signaling

AI Masyuk, TV Masyuk, PL Splinter, BQ Huang… - Gastroenterology, 2006 - Elsevier
Background & Aims: Cholangiocytes have primary cilia extending from the apical plasma
membrane into the ductal lumen. While the physiologic significance of cholangiocyte cilia is …

Cilia and centrosomes: a unifying pathogenic concept for cystic kidney disease?

F Hildebrandt, E Otto - Nature Reviews Genetics, 2005 - nature.com
Cystic kidney diseases are among the most frequent lethal genetic diseases. Positional
cloning of novel cystic kidney disease genes revealed that their products (cystoproteins) are …

Autosomal recessive polycystic kidney disease: a hepatorenal fibrocystic disorder with pleiotropic effects

EA Hartung, LM Guay-Woodford - Pediatrics, 2014 - publications.aap.org
Autosomal recessive polycystic kidney disease (ARPKD) is an important cause of chronic
kidney disease in children. The care of ARPKD patients has traditionally been the realm of …