All the necessary research information in one place

Every help is a great motivation and a boost of energy for us, thanks to which we continue. You can help too!

01

PROGRESS OF RESEARCH

Read about the current state of scientific knowledge in the field of rare human diseases.

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02

RESEARCH TARGET

Our vision, research goals, technologies used and the role of Asgent.

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03

SCIENTIFIC BOARD

People who cover Angelman syndrome research.

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GLOSSARY OF TERMS

Explanation of the most frequently used terms in connection with rare diseases.

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01

The course of the research clearly in several points

We wasted no time, because time is the most valuable asset we have, and we reached out to the world’s leading scientists and doctors and, of course, patients and their families. Together, we started research aimed at finding a cure for our son’s rare disease at the Czech Centre for Phenogenomics – one of the largest, most modern and most powerful of its kind in the world.

The Centre replicates a number of rare human diseases through advanced rodent models on which it conducts preclinical testing to discover a potential cure. This centre shows that we are witnessing major advances in human knowledge that could lead to cures for previously incurable diseases. Sometime potential cures are hidden behind layers of research that need to be thoroughly uncovered. we are moving closer to finding a possible cures every day.

In this respect, it is very important for us to obtain new mouse models of human genetic disorders using specific methods – such as the CRISPR/Cas method together with gene silencing – to pave the way for faster and more accurate genetic work. Science is making incredible strides, and ongoing research will help scientists find tools and mechanisms for potential future treatments for hundreds, if not thousands, of genetic diseases.

How did it begin

Imagine the moment when you are told that your child is terminally ill, and that he or she will be dependent upon you for the rest of their life. It was a feeling like we would never again be able to breathe. We will never forget it!

And yet, just four months after being told the diagnosis, we were able to breathe, laugh, enjoy each day and to move on.

“We have found the best way for us to face this reality. Based on our own initiative, basic research into the possible treatments for Angelman Syndrome has begun in the Czech Republic.”

—Founders Rado and Lenka

 

2018 - 02

Establishing cooperation

In 2018, the cooperation between the non-profit organization ASGENT and CCP was established.

This was immediately followed by identifying and defining what would be researched. The aim of the research is to study selected genes and biological processes involved in the formation of Angelman syndrome. Understanding this may be important for finding treatments for genetic defects.

 

2018 - 04

Starting research

The same year, the CCP Review – a summary of the issues in the literature and the current level of knowledge, followed – A proposal of possible research paths and possible new approaches, immediately after the start of the research.

2018 - 09

First phase of research

For research, several unique research mouse models of a human genetic defect have been created using the CRISPR/Cas method.

2019

Second phase of research

Research continues by examining the phenotypic manifestations of Angelman syndrome. We want to better understand the connections between clinical manifestations and the role of individual genes in the area responsible for the development of Angelman syndrome.

ASGENT supported the research with the amount of CZK 500,000.

2020

Scientists have developed new materials. A possible therapeutic procedure is being worked on.

Asgent supported the research with the sum of CZK 1 million.

2021

Angelman syndrome is included as one of the model disorders in the new AV21 Strategy program – “Gene and precision therapy – a new hope in the treatment of human diseases”, which aims to accelerate the transfer of experimental gene therapy approaches to clinical research.

2022
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Main research goals, vision for the future

The aim of the research is to study selected genes and their biological processes involved in the development of Angelman syndrome. If we understand how they work we may discover treatments for genetic defects.

The disease we are studying is very rare and therefore investors and big pharmaceutical companies do not see a chance of profit in developing a cure. We are trying to show the world our point of view by showing them life through the eyes of a patient suffering from a rare disease. It is precisely because we were not getting the help we needed that we decided to take matters into our own hands.

Believe us, no one is more motivated to make progress than the families affected by this disease who are fighting an insidious illness. “Hope is not the belief that something will turn out well, but the certainty that something make sence, no matter how it turns out.” – Václav Havel

The aim of the research is to study selected genes and biological processes involved in the formation of Angelman syndrome. Understanding this may be important for finding treatments for genetic defects.

THE PLACE WHERE THE RESEARCH TAKES PLACE

Radislav Sedláček, Ph.D., Doc.Dr., Director of the large national research infrastructure of the Czech Centre for Phenogenomics, Institute of Molecular Genetics, classified Angelman syndrome as a subject of research from several perspectives.

ANGELMAN SYNDROME FROM THE PERSPECTIVE OF GENETICS

In the case of Angelman syndrome, the genetic defect is located on the 15th chromosome in the region (15q11-13). This is also the region responsible for Prader-Willi Syndrome, where several genes and biological processes are still not well understood. The brain is physiologically fine in Angelman syndrome. It’s not damaged in any way. The neurons are developed, but because of a missing protein such as UBE3A, which is not naturally produced, it does not function as it should.

WE SUPPORT TOP SCIENTISTS

The science is moving in leaps and bounds in this area. We have met excellent scientists who are also excellent people. We are grateful to be able to be there and support research activities. For this reason, we have established the Association of Gene Therapy to actively support these activities. Help us in any way you can think of, you will be supporting world-class research with enormous potential.

FIRST PHASE OF RESEARCH

First of all, it is important to obtain new mouse models of human genetic defects using the CRISPR/Cas method. This is currently underway and it is expected that they would start to be investigated in the first half of 2019.

So far, similar models have only been partially investigated, but the Czech Centre for Phenogenomics has a specially developed platform, one of the best in the world, in which up to 1000 parameters from major physiological systems can be obtained, thus providing a comprehensive picture of the function of any gene or biological process.

TECHNOLOGY

The phenomenon of gene silencing, a biological phenomenon that is also responsible for the dysfunctional regulation of gene activity, will be the most studied. Here, too, the research facility will use new molecular tools, such as CRISPR/Cas technology, which overall opens up faster and more precise possibilities for work in genetics.

Answering the questions this research is asking early on may help to find tools and mechanisms for future treatments for some of the hundreds of diseases, not just genetic diseases.

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Scientific board

Doc.Dr. Radislav Sedláček, PhD.

Doc.Dr. Radislav Sedláček, PhD.

Member of the BIOCEV Board, Head of the Functional Genomics Programme, Head of Infrastructure of the Czech Centre for Phenogenomics (CCP). Radislav Sedláček is one of the leading Czech experts in the field of transgenic and genetically modified organisms, especially genetically modified mouse models.

R. Sedláček came to the Institute of Molecular Genetics, v. v. i., after a long-term stay abroad and his main task was to establish transgenic technologies in the Czech Republic so that we would not depend only on foreign workplaces.

prof. MUDr. Jiří Forejt, DrSc.

prof. MUDr. Jiří Forejt, DrSc.

In his research, Jiří Forejt uses the mouse genome as a model for understanding the laws of the origin of new species and for studying the heredity of human development and human diseases.

Mouse inbred strains PWD/Ph and PWK/Ph, which were created in his laboratory more than 30 years ago, are used in genetic laboratories in countries on four continents, and his chromosomal replacement strains, taken from The Jackson Laboratory in the USA, serve as a biomedical model for systems genetics.

MUDr. Alena Zumrová, Ph.D.

MUDr. Alena Zumrová, Ph.D.

She has been working since 1982 at the Department of Paediatric Neurology, 2nd Faculty of Medicine, Charles University in Prague and Motol University Hospital in Prague.In 2008, she founded the Centre for Hereditary Ataxia at Motol University Hospital with a nationwide scope, which in 2018 received the status of a reference centre within the European Reference Network for Rare Neurological Diseases (ERN-RND). She focuses on neurodevelopmental, neurometabolic, neurodegenerative and neurogenetic rare diseases.

Our team

Ing. Radoslav Hajgajda

Ing. Radoslav Hajgajda

Chairman and co-founder of the Association, Oliver's dad. Initiator of basic research on rare diseases.

Ing. Lenka Matějková

Ing. Lenka Matějková

Co-founder of the Association, Oliver's mom. PR communication, contact for journalists.

Mgr. Tatiana Aghová, Ph.D.

Mgr. Tatiana Aghová, Ph.D.

I am a scientist and curator of the exhibition The Gene Age (www.doba-genova.cz). I enjoy working in the molecular lab as much as popularizing science. I am convinced that basic research is of great importance to our lives,even if it is not always apparent at first sight. That is why it is a great honour to be part of the Gene Therapy Association and to bring science and public communication together.

If you have any questions, please get in touch. We look forward to hearing from you.

Contact
04

Glossary of terms

It slows down development and causes various problems with speech, balance and intellect. Around 100 children in the Czech Republic have been diagnosed with this condition. It manifests itself initially with delayed development, later with reduced intellect, difficulty in walking, very often epilepsy and sleep disorders

Seizures are often part of life for an “Angelman” like Oliver. The disease was originally named after Dr. Angelman. The children who suffer from the disease can usually be found smiling and exhibiting positive behavior.

 

This method, credited to American scientist Jenifer Doudna, was discovered in 2012. It was a major discovery that seemed to kick-start genetic research to a much higher level. The basis of the method is the use of the immune system of bacteria. Even bacteria have their enemies. These are viruses, for example. The way a bacterium’s defence system works is that if a virus attacks it, the bacterium is able to identify the virus, unwind it and cut its DNA. Because the virus has damaged DNA, it can no longer reproduce. The bacterium “remembers” viruses that have attacked it in the past, that is, it writes parts of the DNA of viruses that have attacked the bacterium in the past into its CRISPR “memory”.

It means that its genetic makeup has been purposefully altered in ways that do not normally occur in nature. Most often GMOs are prepared by artificially introducing into a certain organism a gene isolated from another species, which leads to overcoming the so-called natural reproductive barrier. In practice, this looks like, for example, a gene from a human being is transferred into a bacterium or a gene from a bacterium into a plant. This way of changing genetic information is called transgenesis, and organisms modified in this way are called transgenic. In contrast to breeding, which was experimented with, for example, by the founder of genetics, Gregor Johann Mendel, transgenesis leads to organisms with completely new properties that cannot be induced by classical genetic methods. Genetic modification is used in research, the results of which lead to a better understanding of the nature of certain diseases and to the production of more effective drugs and improved treatments.

Gene therapy is the next generation of drugs that targets the underlying cause of a genetic disease. It has the potential to provide patients with a breakthrough clinical benefit and significantly improve their quality of life. These ground-breaking single-dose therapies will impact the way we treat patients globally over the next few years, potentially reducing the burden on patients of the need for daily administration and thereby contributing to the efficiency of the healthcare system.

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