Lab-grown ‘mini-kidneys’ unlock secrets of a rare disease

A microscope image of the kidney tissue is used to uncover a medical secret about complex sclerosis. Credit: Cell Research

Researchers have uncovered a medical secret in a highly misunderstood disease by identifying which viruses cause tumors in patients with meningitis (TSC). As described in Cell Reportthey do this by producing kidney cells, or “small kidneys” that grow from the human body.

“Cells that originate from cancer-related tumors have been a mystery for many years,” said lead author Dr. Bill Stanford, a senior scientist at Ottawa Hospital and a professor at the University of Ottawa. “Our findings may help identify potential therapeutic targets for this disease challenge.”

TSC is a rare disease that causes tumors in the skin, brain, kidneys, heart or lungs. TSC cancer is highly variable, developing in children or adults with a range of symptoms ranging from mild to life-threatening and often includes seizures. kidney problems. There is no treatment and treatment options are limited.

“Kidney disease is the leading cause of death for TSC patients, about 60 to 80 per cent of patients are diagnosed with kidney cancer, usually declining. kidney function sometimes it causes severe bleeding, ”said President Dr. Adam Pietrobon, MD-Ph.D. student at Ottawa Hospital and University of Ottawa. “There are not enough lab samples to study how TSC affects the kidneys, so we did. one ourselves. “

TSC occurs through mutations in the TSC1 or TSC2 gene. For many patients, these mutations only develop during adulthood or early life rather than inheritance from their parents. This makes TSC a difficult disease to diagnose. While laboratory researchers often use animals to diagnose human diseases, no animal product has captured the effects of TSC on the kidneys.

TSC cancer in the kidneys is confusing to professionals because they vary in size, cellular makeup and physical appearance, even in the same patient. The cause of this difference is unknown, and makes treatment challenging.

To better understand the impact of TSC on the kidneys, the team developed 3-D kidney tissue in the laboratory from human genetically engineered cells to obtain TSC1 or TSC2 mutations. Known as organoids, these tiny, simple kidney cells have biological data similar to TSC tumors found in copyright. Then the researchers took over Single cells from these kidney cells and injected into the kidneys of mice, where they grow into human TSC tumors.

Using these genes, the researchers revealed that cells called Schwann Cell Precursors are where TSC tumors start in the kidney. They also found that this single mutation affects the proliferation of different cells, which explains the difference in kidney disease in the same person.

“Not only can these‘ small kidneys ’help us understand this disease, they can also be used to try new treatment options,” Dr. Pietrobon said.

Research in human kidney cells reveals the goal of preventing chronic kidney damage

Learn more:
Adam Pietrobon et al, Renal organoid modeling of tuberous sclerosis complex describes weak features arising from differentiated developmental processes, Cell Report (2022). DOI: 10.1016 / j.celrep.2022.111048

Provided by Ottawa Hospital

hint: Lab-size ‘small-kidneys’ unravel the mystery of a rare disease (2022, July 6) restored July 6, 2022 from -mini-kidneys-secret- rare-disease.html

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