Glomerular and proximal tubule cysts as early manifestations of Pkd1 deletion

Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association
Ali K AhrabiOlivier Devuyst

Abstract

The homozygous deletion of Pkd1 in the mouse results in embryonic lethality with renal cysts and hydrops fetalis, but there is no precise data on the segmental origin of cysts and potential changes associated with polyhydramnios. We used Pkd1-null mice to investigate cystogenesis and analyze the amniotic fluid composition from embryonic day 12.5 (E12.5) to birth (n = 257 embryos). Polyhydramnios was consistently observed from E13.5 in Pkd1(-/-) embryos, in absence of placental abnormalities but with a significantly higher excretion of sodium and glucose from E13.5 through E16.5, and increased cyclic adenosine 3'5-monophosphate (cAMP) levels at E14.5 and E15.5. The Pkd1(-/-) embryos started to die at E13.5, with lethality peaking at E15.5, corresponding to the onset of cystogenesis. The first cysts in Pkd1(-/-) kidneys emerged at E15.5 in mesenchyme-derived segments at the cortico-medullary junction, with a majority of glomerular cysts and fewer proximal tubule cysts (positive for megalin). The cysts extended to ureteric bud-derived collecting ducts (positive for Dolichos biflorus agglutinin lectin) from E16.5. These studies indicate that Pkd1 deletion is associated with a massive loss of solutes (from E13.5) and increased cAMP ...Continue Reading

References

Jul 1, 1976·American Journal of Obstetrics and Gynecology·B H YuenK Staley
Aug 1, 1989·American Journal of Obstetrics and Gynecology·R A Brace, E J Wolf
Jan 1, 1983·The Journal of Histochemistry and Cytochemistry : Official Journal of the Histochemistry Society·F MurataT Muramatsu
Jun 10, 1997·Proceedings of the National Academy of Sciences of the United States of America·O Ibraghimov-BeskrovnayaK W Klinger
Apr 23, 1999·Developmental Dynamics : an Official Publication of the American Association of Anatomists·R GuillaumeM Trudel
Mar 4, 2000·Proceedings of the National Academy of Sciences of the United States of America·K KimM A Arnaout
Oct 11, 2001·Proceedings of the National Academy of Sciences of the United States of America·C BoulterR Sandford
Mar 14, 2002·The American Journal of Pathology·Véronique ChauvetMarie-Claire Gubler
May 8, 2002·Nature Reviews. Molecular Cell Biology·Erik Ilsø Christensen, Henrik Birn
Jul 4, 2002·Human Molecular Genetics·Satoru MutoShigeo Horie
Dec 17, 2003·Kidney International·François JouretOlivier Devuyst
Mar 3, 2004·Nature Medicine·Vicente E TorresVincent H Gattone
Feb 25, 2005·Journal of the American Society of Nephrology : JASN·Xiaofang WangVicente E Torres
Mar 12, 2005·The Journal of Clinical Investigation·Saori NishioToshio Mochizuki
Jun 1, 2005·Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia·Tien-Jen LinCheng-Kuei Chang
Dec 6, 2005·Journal of the Society for Gynecologic Investigation·Cecilia Y Cheung, Robert A Brace
Feb 2, 2006·Molecular and Cellular Biology·Caroline ThiviergeMarie Trudel
Aug 26, 2006·Nature Clinical Practice. Nephrology·Vicente E Torres, Peter C Harris
May 4, 2007·Journal of the American Society of Nephrology : JASN·Ali K AhrabiOlivier Devuyst
May 16, 2009·Clinical Journal of the American Society of Nephrology : CJASN·Vicente E TorresJared J Grantham
Jan 1, 1983·The Journal of Histochemistry and Cytochemistry : Official Journal of the Histochemistry Society·F MurataT Muramatsu

❮ Previous
Next ❯

Citations

Aug 2, 2011·Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association·Olivier DevuystAndreas Serra
Oct 16, 2012·The Journal of Clinical Investigation·Katharina HoppPeter C Harris
Sep 24, 2010·PloS One·Miguel A Garcia-GonzalezTerry Watnick
Apr 5, 2013·PloS One·Corina AndersAdrian S Woolf
Mar 8, 2013·Kidney International·Hester HappéDorien J M Peters
Nov 4, 2016·Nephrology, Dialysis, Transplantation : Official Publication of the European Dialysis and Transplant Association - European Renal Association·Ron T GansevoortUNKNOWN TEMPO 3:4 Investigators
Jan 25, 2011·Biochimica Et Biophysica Acta·Sara TerrynOlivier Devuyst
Jun 25, 2014·Molecular and Cellular Biology·Almira KurbegovicFeng Qian
Oct 11, 2017·Molecular and Cellular Biology·Yu IshimotoMasaomi Nangaku
Apr 3, 2019·The Journal of Pharmacology and Experimental Therapeutics·Stephen C LenhardErding Hu
Oct 3, 2020·Bioengineering & Translational Medicine·Yi HuangEun Ji Chung
Dec 14, 2018·Nature Reviews. Disease Primers·Carsten BergmannVicente E Torres
Aug 5, 2020·Journal of the American Society of Nephrology : JASN·Shohei KuraokaRyuichi Nishinakamura
Dec 2, 2020·International Journal of Molecular Sciences·Maria FragiadakiAlbert C M Ong

❮ Previous
Next ❯

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.