Autosomal dominant polycystic kidney disease: the changing face of clinical management

ACM Ong, O Devuyst, B Knebelmann, G Walz - The Lancet, 2015 - thelancet.com
Autosomal dominant polycystic kidney disease is the most common inherited kidney disease
and accounts for 7–10% of all patients on renal replacement therapy worldwide. Although …

Polycystin channel complexes

O Esarte Palomero, M Larmore… - Annual review of …, 2023 - annualreviews.org
Polycystin subunits can form hetero-and homotetrameric ion channels in the membranes of
various compartments of the cell. Homotetrameric polycystin channels are voltage-and …

[HTML][HTML] Drugs in clinical development to treat autosomal dominant polycystic kidney disease

T Bais, RT Gansevoort, E Meijer - Drugs, 2022 - Springer
Autosomal dominant polycystic kidney disease (ADPKD) is characterized by progressive
cyst formation that ultimately leads to kidney failure in most patients. Approximately 10% of …

Right, left and cilia: How asymmetry is established

RB Little, DP Norris - Seminars in Cell & Developmental Biology, 2021 - Elsevier
The initial breaking of left-right (L–R) symmetry in the embryo is controlled by a motile-cilia-
driven leftward fluid flow in the left-right organiser (LRO), resulting in L–R asymmetric gene …

[HTML][HTML] Advances in autosomal dominant polycystic kidney disease: a clinical review

N Nobakht, RM Hanna, M Al-Baghdadi, KM Ameen… - Kidney Medicine, 2020 - Elsevier
Polycystic kidney disease (PKD) is a multiorgan disorder resulting in fluid-filled cyst
formation in the kidneys and other systems. The replacement of kidney parenchyma with an …

[HTML][HTML] SCF-SKP2 E3 ubiquitin ligase links mTORC1/ER stress/ISR with YAP activation in murine renal cystogenesis

DK Panda, X Bai, Y Zhang… - The Journal of …, 2022 - Am Soc Clin Investig
The Hippo pathway nuclear effector Yes-associated protein (YAP) potentiates the
progression of polycystic kidney disease (PKD) arising from ciliopathies. The mechanisms …

[HTML][HTML] Vasopressin–aquaporin-2 pathway: recent advances in understanding water balance disorders

M Ranieri, A Di Mise, G Tamma, G Valenti - F1000Research, 2019 - ncbi.nlm.nih.gov
The alteration of water balance and related disorders has emerged as being strictly linked to
the state of activation of the vasopressin–aquaporin-2 (vasopressin–AQP2) pathway. The …

Spatiotemporal dynamics and heterogeneity of renal lymphatics in mammalian development and cystic kidney disease

DJ Jafree, D Moulding, M Kolatsi-Joannou… - Elife, 2019 - elifesciences.org
Heterogeneity of lymphatic vessels during embryogenesis is critical for organ-specific
lymphatic function. Little is known about lymphatics in the developing kidney, despite their …

[HTML][HTML] Studying kidney diseases using organoid models

M Liu, A Cardilla, J Ngeow, X Gong… - Frontiers in Cell and …, 2022 - frontiersin.org
The prevalence of chronic kidney disease (CKD) is rapidly increasing over the last few
decades, owing to the global increase in diabetes and cardiovascular diseases. Dialysis …

[HTML][HTML] Molecular pathways involved in injury-repair and ADPKD progression

C Formica, DJM Peters - Cellular Signalling, 2020 - Elsevier
The major hallmark of Autosomal Dominant Polycystic Kidney Disease (ADPKD) is the
formation of many fluid-filled cysts in the kidneys, which ultimately impairs the normal renal …