Polycystic Kidney Disease

Uniquely engineered mouse model for polycystic kidney disease drug development

nephrology preclinical services

With a prevalence of 4 in 10.000, Autosomal Dominant Polycystic Kidney Disease (ADPKD) is a relatively common genetic disorder. It has a profound disease burden on patients due to the formation of many fluid filled cysts in their kidneys. In addition, patients may also suffer from extrarenal complications such as liver cysts, pancreatic cysts, hypertension and cardiovascular abnormalities. In the majority of cases a mutation in Pkd1, the gene coding the polycystine-1 protein, underlies the disease.

To support the preclinical development of new drugs and therapies for ADPKD, InnoSer offers a unique genetically and phenotypically relevant in vivo mouse assay. Multiple well established and validated conditional (Tamoxifen) inducible kidney specific Pkd1 knockout mouse (Pkd1-cKO) models are available to meet your specific requirements in terms of clinical translatability, outcome measures and study duration.

Model options

P10 and P18 are highly demanded models and are well characterized in our pipelines, while P40 options offer even closer to clinics phenotypes.

Model Tamoxifen Cyst characteristics
P10 2 consecutive days from P10 onward Quick progression of cyst formation in the distal segment of the nephron
P18 3 consecutive days from P18 onward Slow progression with cysts in all segments of the nephron
P40 3 consecutive days from P40 onward Slow and reproducible cyst formation in the proximal part of the nephron
P40 slow A low dose at 3 consecutive days from P40 Very slow cyst formation, taking 6-8 months. At 10-13 months this model closely resembles the human phenotype

 

Key readouts

  • Body weight (monitored daily)
  • Kidney weight
  • Kidney weight to body weight ratio
  • Urea measurement
  • Histopathology: cystic index and pathological lesions
  • Ultrasound imaging to determine kidney volume

Additional advantages

  • Multiple well established models
  • AAALAC accredited facilities
  • Organ and blood sampling for additional tailored analysis
Dorien Peters

Developed by Prof. Dr. Dorien Peters of the LUMC

ADPKD mouse model is developed by a collaborative project with Dr. Peters from Leiden University Medical Center (LUMC) in lead.

Also involved in InnoSer’s Scientific Advisory Board, Dr. Peters focuses on insights into the genetic, pathophysiologic and functional mechanisms of inherited disorders.

kidney ultrasound imaging

Powered by ultrasound

In vivo imaging has become a valuable tool for collecting mid study data particularly in long term animal studies. These non-invasive methods can be applied with high sensitivity and in real time. With InnoSer’s imaging capabilities, track your lead compound effects on cyst growth easily. Read more >>

adpkd mouse cysts

Smart pathological assessment

Histopathological assessments are standard parts of our ADPKD model readouts. Thanks to smart algorithms being trained to identify cysts in an automated manner, these can be offered in an efficient way, also with possibilities of long term and secure cloud storage of the images. Read more >>

Laura Blockken

Scientific experts to guide you

An expert team of scientists with vast experience in our ADPKD model helps you choose the right model options and set up optimal study designs for your nephrology projects. Curating the preclinical testing of your lead compounds with a deep understanding in the field is your solution to accelarating your drug development.

Would you like more information?

kidney disease resources

Here you can request InnoSer’s ADPKD leaflet that contains sample data to learn about the use of the platform for preclinical drug development.

Interested In InnoSer’s ADPKD Model?

Get in touch with us.