[HTML][HTML] Molecular pathogenesis of ADPKD: the polycystin complex gets complex

ACM Ong, PC Harris - Kidney international, 2005 - Elsevier
Molecular pathogenesis of ADPKD: The polycystin complex gets complex. Autosomal-
dominant polycystic kidney disease (ADPKD) is one of the most common human monogenic …

[HTML][HTML] Cilium-generated signaling and cilia-related disorders

J Pan, Q Wang, WJ Snell - Laboratory investigation, 2005 - Elsevier
Biologists have long known that humans experience their environment through cilia. Light,
odorant, and sound perception depend on these microtubule-filled, complex organelles …

[HTML][HTML] Altered trafficking and stability of polycystins underlie polycystic kidney disease

Y Cai, SV Fedeles, K Dong… - The Journal of …, 2014 - Am Soc Clin Investig
The most severe form of autosomal dominant polycystic kidney disease occurs in patients
with mutations in the gene (PKD1) encoding polycystin-1 (PC1). PC1 is a complex polytopic …

[HTML][HTML] Endocytic trafficking of membrane-bound cargo: a flotillin point of view

M Meister, R Tikkanen - Membranes, 2014 - mdpi.com
The ubiquitous and highly conserved flotillin proteins, flotillin-1 and flotillin-2, have been
shown to be involved in various cellular processes such as cell adhesion, signal …

Development of polycystic kidney disease in juvenile cystic kidney mice: insights into pathogenesis, ciliary abnormalities, and common features with human disease

LA Smith, NO Bukanov, H Husson… - Journal of the …, 2006 - journals.lww.com
Significant progress in understanding the molecular mechanisms of polycystic kidney
disease (PKD) has been made in recent years. Translating this understanding into effective …

Polycystin-1 C-terminal tail associates with β-catenin and inhibits canonical Wnt signaling

M Lal, X Song, JL Pluznick, V Di Giovanni… - Human molecular …, 2008 - academic.oup.com
Abstract Polycystin-1 (PC1), the product of the PKD1 gene mutated in the majority of
autosomal dominant polycystic kidney disease (ADPKD) cases, undergoes a cleavage …

Essential role of cleavage of Polycystin-1 at G protein-coupled receptor proteolytic site for kidney tubular structure

S Yu, K Hackmann, J Gao, X He… - Proceedings of the …, 2007 - National Acad Sciences
Polycystin-1 (PC1) has an essential function in renal tubular morphogenesis and disruption
of its function causes cystogenesis in human autosomal dominant polycystic kidney disease …

Toxic tubular injury in kidneys from Pkd1-deletion mice accelerates cystogenesis accompanied by dysregulated planar cell polarity and canonical Wnt signaling …

H Happé, WN Leonhard, A van der Wal… - Human molecular …, 2009 - academic.oup.com
Autosomal dominant polycystic kidney disease (ADPKD) is characterized by large fluid-filled
cysts and progressive deterioration of renal function necessitating renal replacement …

[HTML][HTML] Fibrosis and progression of autosomal dominant polycystic kidney disease (ADPKD)

J Norman - Biochimica et Biophysica Acta (BBA)-Molecular Basis …, 2011 - Elsevier
The age on onset of decline in renal function and end-stage renal disease (ESRD) in
autosomal polycystic kidney disease (ADPKD) is highly variable and there are currently no …

[PDF][PDF] Детская нефрология

МС Игнатова, ЮЕ Вельтищев - Руководство для врачей. М …, 2011 - pediatriajournal.ru
111 МС Игнатова креатинин крови 540 мкмоль/л, метаболический ацидоз (pH 7, 27,
рO2 96 mm Hg, рCO2 29, 7 mm Hg, ВЕ–11, 7 мэкв/л), оптическая плотность мочи 1003 …