Emerging therapies for autosomal dominant polycystic kidney disease with a focus on cAMP signaling

X Zhou, VE Torres - Frontiers in Molecular Biosciences, 2022 - frontiersin.org
Autosomal dominant polycystic kidney disease (ADPKD), with an estimated genetic
prevalence between 1: 400 and 1: 1,000 individuals, is the third most common cause of end …

Role of the polycystins as mechanosensors of extracellular stiffness

EA Nigro, A Boletta - American Journal of Physiology-Renal …, 2021 - journals.physiology.org
Polycystin-1 (PC-1) is a transmembrane protein, encoded by the PKD1 gene, mutated in
autosomal dominant polycystic kidney disease (ADPKD). This common genetic disorder …

[HTML][HTML] The tryptophan-metabolizing enzyme indoleamine 2, 3-dioxygenase 1 regulates polycystic kidney disease progression

DT Nguyen, EK Kleczko, N Dwivedi, MLT Monaghan… - JCI insight, 2023 - ncbi.nlm.nih.gov
Autosomal dominant polycystic kidney disease (ADPKD), the most common monogenic
nephropathy, is characterized by phenotypic variability that exceeds genic effects …

Restoration of atypical protein kinase C ζ function in autosomal dominant polycystic kidney disease ameliorates disease progression

M Akbari, JD West, N Doerr, KR Kipp… - Proceedings of the …, 2022 - National Acad Sciences
Autosomal dominant polycystic kidney disease (ADPKD) affects more than 500,000
individuals in the United States alone. In most cases, ADPKD is caused by a loss-of-function …

A brief review on the regulatory roles of MicroRNAs in cystic diseases and their use as potential biomarkers

LM Ruiz-Manriquez, SJ Ledesma Pacheco… - Genes, 2022 - mdpi.com
miRNAs are small endogenous conserved non-coding RNA molecules that regulate post-
transcriptional gene expression through mRNA degradation or translational inhibition …

Factors associated with early-onset intracranial aneurysms in patients with autosomal dominant polycystic kidney disease

Y Ushio, H Kataoka, H Akagawa, M Sato… - Journal of …, 2024 - Springer
Background Recently, the importance of attribute-based medicine has been emphasized.
The effects of early-onset intracranial aneurysms on patients can be significant and long …

The cellular pathways and potential therapeutics of Polycystic Kidney Disease

T Richards, K Modarage, SA Malik… - Biochemical Society …, 2021 - portlandpress.com
Polycystic Kidney Disease (PKD) refers to a group of disorders, driven by the formation of
cysts in renal tubular cells and is currently one of the leading causes of end-stage renal …

[HTML][HTML] Loss of Polycystin-1 causes cAMP-dependent switch from tubule to cyst formation

JK Scholz, A Kraus, D Lüder, K Skoczynski, M Schiffer… - Iscience, 2022 - cell.com
Autosomal dominant polycystic kidney disease is the most common monogenic disease that
causes end-stage renal failure. It primarily results from mutations in the PKD1 gene that …

The carboxy‐terminus of the human ARPKD protein fibrocystin can control STAT3 signalling by regulating SRC‐activation

C Dafinger, AM Mandel, A Braun… - Journal of cellular …, 2020 - Wiley Online Library
Autosomal recessive polycystic kidney disease (ARPKD) is mainly caused by variants in the
PKHD1 gene, encoding fibrocystin (FC), a large transmembrane protein of incompletely …

Defects of renal tubular homeostasis and cystogenesis in the Pkhd1 knockout

JC Fox, ST Hahnenstein, F Hassan, A Grund, D Haffner… - Iscience, 2024 - cell.com
20 Loss of PKHD1-gene function causes autosomal recessive polycystic kidney disease 21
(ARPKD) characterized by bilateral severely enlarged kidneys and congenital liver fibrosis …