According to a recent article in MedicalXpress, scientists have discovered a possible method of treating amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). The study was also published in Science Advances in February 2024.
FTD follows a close second to ALS with respect to dementia. However, both disorders occur as a result of degeneration of the nervous tissue, the mechanisms of which still remain unclear.
There is a distinction between FTD and ALS as opposed to Alzheimer’s. Unlike the latter disease, the former two disorders do not carry biomarkers showing that a patient exhibits shrinkage in their frontotemporal lobe.
A New Pathological Method
Recently Dr. Yun-Ru Chen and the GRC team, working with short chains of amino acids called synthetic peptides, discovered a pathological mechanism of neuronal degeneration. The doctor’s laboratory has worked with neurodegenerative diseases related to protein misfolding for years. The disease affects the central nervous system. Cells are no longer able to function and eventually die.
The disorders have no cure and worsen over time. They may be the result of a tumor or stroke or may be genetic. The mechanisms of a disease are usually activated by the underlying causes which, if controlled, could prevent the disorder.
In addition, the researchers discovered that a class of sugar called disaccharide may expand neuronal survival as well as reduce degeneration.
About ALS and FTD
Both disorders usually occur to middle-aged individuals. Symptoms of ALS are paralysis and muscle atrophy caused by motor neurons deteriorating in the motor cortex, spinal cord, and brainstem.
ALS is often called Lou Gehrig’s disease after the famous baseball star. The well-known scientist Dr. Stephen Hawking battled ALS at each challenging progression of the disease.
FTD patients exhibit changes in behavior and impairments to speech resulting from deterioration of the neurons in temporal and frontal lobes. Hollywood actor Bruce Willis, known for his action movies, retired in 2022 as a result of his FTD diagnosis.
Although the clinical symptoms of FTD and ALS are different, both diseases share genetic variations and pathological features.
Scientists have found that over 90% of ALS and about 70% of FTD cases are sporadic. This creates difficulty when attempting to analyze the genetic variations associated with the disease.
They did find, however, that in case studies of family members, the genetic mutation that was most common for both disorders originated from the G4C2 sequence2 of the C9orf72 gene.
The discovery that repeat expansions in the C9orf72 gene are a frequent cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) has revolutionized our understanding of these diseases.
