What is ALS?


Amyotrophic lateral sclerosis (ALS), first mentioned in 1874 by the French neurologist Jean-Martin Charcot, is one of the most severe neurodegenerative diseases that affects humans. ALS causes progressive and irreversible damage to the motor neurons - the nerve cells that are responsible for muscle movements. The two motor neurons that are affected by ALS are the first motor neuron, which is located in the cerebral cortex, and the second motor neuron, which is located in the spinal cord and whose axons attach to the skeletal muscles. If these nerve cells are damaged, the muscles are no longer sufficiently innervated.

The first motor neuron is also called the upper motor neuron because it is superior to the second, lower motor neuron. If this first motor neuron fails, it leads to spastic paresis (involuntary muscle twitching, cramping, or paralysis). If the second motor neuron is damaged, the muscles go limp and the result is atrophy and weakness in the arms, legs and respiratory muscles. The first symptoms of the disease occur in different places, depending on the patient. Muscle atrophy and weakness sometimes appear first in the hand and forearm muscles of one side of the body, before extending to the other side of the body and to the legs. A small part of patients experience the first symptoms when talking, chewing, and swallowing. This type of progression of the disease is referred to as "bulbar".

In Germany alone, about 8,000 patients are affected by this disease and about 2,000 die each year. Despite extensive research, the causes of ALS are still not fully understood and the therapeutic options are limited only to the treatment of the symptoms. There is no cure. The average survival time is 4-5 years. To combat the continued lack of public perception regarding ALS, it is imperative to not only open people's eyes to this disease but also to develop new therapeutic strategies.

Why facALS?


faceALS, a nonprofit foundation established on May 22, 2017, was brought about by the faceALS initiative, which was launched two years earlier by Karl-Heinz Zacher, Prof. Christian Schreiber and Dr. Heiko Ott. The reason for establishing the foundation was the personal experience of the initiators with the untreatable and incurable disease of amyotrophic lateral sclerosis (ALS). The name "faceALS" was chosen deliberately by the late Nina Zacher, to give the disease that ultimately caused her death a face and to sensitize society with numerous publicity articles and blogs about the subject.

The current state of scientific research gives very little reason to hope that the approximately 350,000 affected people worldwide will receive tolerable and dignified treatment or therapy in the foreseeable future. Increased public awareness of the sufferers and their relatives, as well as financial support for scientific research, is urgently necessary to combat this incurable degenerative disease of the motor nervous system and its accompanying symptoms.

The purpose of the foundation is research in the fields of toponomics, proteomics, and genomics, as well as the scientific implementation of the generated data and insights. The foundation also focuses on public health and education, by raising funds exclusively and directly for the promotion of these charitable causes. The faceALS Foundation is operationally as well as promotionally active; in other words, the foundation carries out its own projects, but also awards funding, based on application and positive assessment.

How to decode the ALS toponome:


To achieve its goals, the faceALS Foundation relies on several pillars. One of them is the foundation's own laboratory, which has a unique and innovative scientific approach, that was developed in Magdeburg by the research group "Molecular Pattern Recognition Research" and Walter Schubert (former university lecturer at the Otto von Guericke University in Magdeburg, visiting professorships for toponomics in Germany and abroad) and validated by top-level publications.

The term toponomics was coined using the naming convention of the biological research areas of genomics and proteomics. The toponome describes the spatial protein networks of cells and tissues. These protein networks are inherently hierarchically structured and are mapped using toponome analysis. It was shown that the networks are controlled by so-called "lead proteins". If these proteins are blocked or down-regulated, the spatial protein network structure breaks down, which leads to a collapse of the pathological networks.

A globally unique microscopy technique known as Imaging Cycler Microscopy (ICM, ToposNomos Ltd.) is available to us as a method for collecting toponomic data. This robotic technology gives us the ability to decode the ALS toponome, recognize pathological mechanisms, and ultimately derive a therapy for ALS. We assume that ALS is a multifactorial disease involving the immune system. The creation of an ALS toponome, while incorporating immunological components, is the most promising step for us, as the ICM is a process that alters neither the shape of the natural biological structure, or spatial arrangement, nor the proteins themselves. In this way, we can obtain hypothesis-free data that, for the first time, provides us with an explanation for the genesis and pathological processes of ALS.

faceALS-Stiftung
Schriftliches Grußwort der Schirmherrin
Barbara Stamm, MdL
Präsidentin des Bayerischen Landtags

„Wer hofft, hat schon gesiegt und siegt weiter.“

Dieser Satz von Jean Paul bringt eine grundlegende Wahrheit des menschlichen Lebens zum Ausdruck. Und Sie, die Sie als Betroffene, Angehörige, Forschende, Therapeuten oder Ärzte der Krankheit ALS begegnen, erleben Tag für Tag immer wieder aufs Neue den tieferen Sinn dieser Worte.

Den unschätzbaren Wert von Hoffnung hat die Stiftung faceALS schon früh erkannt. Aus einer zunächst privaten Initiative durch Betroffene und Angehörige ist längst etwas Großartiges entstanden. Hier wird auf vielfältige Weise Mut gemacht und dadurch Hoffnung gespendet. Die Stiftung inspiriert, sie vernetzt und sie investiert! Sie führt bereits vorhandene Ansätze der Forschung weiter, um so die Perspektive für die Betroffenen im Kampf gegen die bisher unheilbare Krankheit zu ändern. Dass hierbei nationale und internationale Partner wie auch die Pharmaindustrie mit einbezogen werden ist Grundvoraussetzung und dringlicher Appell für eine vertrauensvolle, in die Zukunft gerichtete Spitzenforschung zum Nutzen aller.

Neben der unverzichtbaren Forschung ist es aber auch wichtig, das gesellschaftliche Bewusstsein zu schärfen. Das heißt sich zu vergegenwärtigen, was es bedeutet, sich mit den Ungewissheiten eines bedrohten Lebens durch ALS zurechtzufinden: Sensibilität wird hier vor allem durch persönliche Geschichten geschaffen, die der Krankheit ein Gesicht geben. Menschen, die sich von ihrem schrecklichen Schicksal so wenig wie möglich haben beeinflussen lassen. Tapfere Kämpferinnen und Kämpfer, die bis zum Schluss durch ihr ermutigendes Beispiel und durch ihre unerschütterliche Präsenz bei so vielen anderen Spuren hinterlassen haben. Diese Spuren gilt es, in unseren Herzen zu bewahren. Ihren Mut und ihren (Kampfes-)Willen gilt es weiterzutragen – damit Menschen, die ein ähnliches Schicksal teilen, keine Angst haben, sondern lernen mit der Angst umzugehen.

Deshalb unterstütze ich als Schirmherrin die Stiftung faceALS aus tiefster Überzeugung und von ganzem Herzen.
Barbara Stamm
Barbara Stamm
Präsidentin des Bayerischen Landtags