Related Experiment Video

Updated: Aug 7, 2026

Use of Ultra-high Field MRI in Small Rodent Models of Polycystic Kidney Disease for In Vivo Phenotyping and Drug Monitoring
07:35

Use of Ultra-high Field MRI in Small Rodent Models of Polycystic Kidney Disease for In Vivo Phenotyping and Drug Monitoring

Published on: June 23, 2015

Understanding pathogenic mechanisms in polycystic kidney disease provides clues for therapy

Peter C Harris1, Vicente E Torres

  • 1Mayo Clinic College of Medicine, Rochester, Minnesota 55905, USA. harris.peter@mayo.edu

Current Opinion in Nephrology and Hypertension
|June 16, 2006
PubMed
Abstract

Insights

Polycystic kidney diseases stem from defective proteins in kidney cilia. Targeting downstream cellular changes offers promising therapeutic strategies currently in patient trials.

Area of Science:

  • Nephrology
  • Genetics
  • Cell Biology

Background:

  • Polycystic kidney diseases (PKDs) are inherited disorders characterized by kidney cyst formation.
  • These conditions arise from genetic defects affecting specific proteins within kidney cells.

Purpose of the Study:

  • To review the common pathogenesis of polycystic kidney diseases.
  • To explore the role of identified defective proteins in disease development.
  • To determine how this knowledge informs potential therapeutic strategies.

Main Methods:

  • Review of scientific literature on polycystic kidney diseases.
  • Analysis of protein localization and function in relation to cilia.
  • Examination of cellular signaling pathways implicated in PKDs.

Main Results:

  • Defective proteins in PKDs are localized to primary cilia/basal body.
  • Phenotypes are often linked to impaired ciliogenesis.
  • Polycystins' role in ciliary mechanosensation and cellular signaling (Ca2+, cAMP) identified.

Conclusions:

  • PKDs involve defects in proteins crucial for kidney cilia function.
  • While primary defects are uncorrectable, downstream cellular pathways can be targeted.
  • Therapeutic agents targeting these pathways are in clinical trials, marking a new phase in PKD research.

More Related Videos

Implementing Patch Clamp and Live Fluorescence Microscopy to Monitor Functional Properties of Freshly Isolated PKD Epithelium08:46

Implementing Patch Clamp and Live Fluorescence Microscopy to Monitor Functional Properties of Freshly Isolated PKD Epithelium

Published on: September 1, 2015

Related Concept Videos

Pharmacogenomics: Identification of New Drug Targets01:29

Pharmacogenomics: Identification of New Drug Targets

Advances in genomics have profoundly influenced drug discovery by increasing both the speed and accuracy of pharmaceutical development. Pharmacogenomics, which examines how genetic variation influences drug response, facilitates the identification of novel therapeutic targets and enables patient stratification for personalized treatment. These strategies contribute to improved drug efficacy, minimized adverse effects, and more efficient clinical trial design.Mapping genetic differences...
Nephrons01:10

Nephrons

The kidneys are intricate organs with millions of working units known as nephrons. Each nephron features two major structures: the renal corpuscle, which facilitates blood plasma filtration, and the renal tubule, which handles the glomerular filtrate. Blood supply is directly linked to the nephrons. The renal corpuscle consists of the glomerulus, a capillary network, and the Bowman's capsule, a double-walled epithelial structure that encases the glomerulus. The filtering of blood plasma happens...
Diabetic Nephropathy01:28

Diabetic Nephropathy

Definition Diabetic nephropathy is a chronic kidney complication that results from prolonged hyperglycemia.Prevalence It is the most common cause of chronic kidney disease (CKD) and end-stage renal disease (ESRD) worldwide, affecting up to half of individuals with diabetes.Pathophysiology • Sustained hyperglycemia triggers multiple hemodynamic and metabolic changes in the kidney. • Early in the disease, increased renal blood flow and glomerular hyperfiltration occur due to afferent arteriolar...
Nephrotic Syndrome II : Assessment and Medical Management01:26

Nephrotic Syndrome II : Assessment and Medical Management

IntroductionNephrotic syndrome is a kidney disorder marked by excessive protein loss in the urine, leading to various systemic complications. This condition often results from damage to the glomeruli—the kidney's filtering units—causing proteinuria, low blood protein levels, and fluid retention. Understanding the assessment, diagnosis, and management of nephrotic syndrome is essential for effective treatment and prevention of further kidney damage.AssessmentPatient History: Document any history...
Acute Kidney Injury V: Interprofessional Care01:20

Acute Kidney Injury V: Interprofessional Care

Acute Kidney Injury (AKI) requires a collaborative healthcare approach to restore renal function and prevent complications. Essential management strategies involve monitoring fluid and electrolyte balance, adjusting medications, initiating dialysis when necessary, and providing nutritional support.Fluid and Electrolyte ManagementFluid Monitoring: Regularly monitoring body weight, central venous pressure, and urine output helps detect fluid imbalances early. Patient intake and output are...
Renal Corpuscle01:20

Renal Corpuscle

The glomerulus and Bowman's capsule are two essential components of the nephron, which is the functional unit of the kidney. These microscopic structures play a critical role in the process of blood filtration to produce urine.
Glomerulus: Structure and Function
The glomerulus is a tiny, intricate network of capillaries located at the beginning of the nephron. It's enveloped by the Bowman's capsule and receives its blood supply from an afferent arteriole, which divides into numerous capillaries...