Korean Researchers Discover Genes for Treating Dementia and Lou Gehrig's Disease

Professor Jeonghoon Lim's Team at UNIST

Human brain. Stock photo. Not related to the article.

[Asia Economy Reporter Kim Bong-su] Domestic researchers have newly discovered a neuroprotective gene that suppresses degenerative brain diseases such as dementia and Lou Gehrig's disease.

Ulsan National Institute of Science and Technology (UNIST) announced on the 30th that Professor Lim Jeong-hoon's team from the Department of Life Sciences discovered the gene ‘ZNF598,’ which suppresses degenerative brain diseases like Lou Gehrig's disease and frontotemporal dementia, and elucidated a new molecular biological principle of neuroprotection.

This gene was found to remove toxic protein translation products within the nerve cells of Lou Gehrig's disease patients, thereby inhibiting cell death. The research results are expected to become a new key for effective early diagnosis of degenerative brain diseases and the development of fundamental brain disease treatments.

The research team activated the ZNF598 gene to suppress the death of nerve cells derived from Lou Gehrig's disease patients. ZNF598 performed this function through the protein translation quality control pathway. Protein translation quality control is the process of recognizing and degrading abnormally produced translation intermediates. When ribosomes responsible for protein translation stall during an abnormal translation process (ribosome stalling), the protein translation quality control pathway is activated, and the translation products are separated from the ribosome and degraded. According to the research results, toxic proteins in Lou Gehrig's disease induce ribosome stalling.

Activating the identified gene suppresses the death of nerve cells derived from patients with Lou Gehrig's disease.

Translation is the process by which the basic structure of proteins is synthesized from the genetic code carried by mRNA. Ribosomes connect each amino acid corresponding to the nucleotide sequence of mRNA to create the basic protein structure, the translation product. It is known that if incorrect genetic information is translated into toxic proteins during this process, degenerative brain diseases such as Lou Gehrig's disease, where nerve cells die, occur.

First author Researcher Park Ju-min explained, “In the process of finding genes that control the cytotoxicity of Lou Gehrig's disease toxic proteins, we discovered a clue that the ZNF598 gene suppresses neurodegeneration and verified this using various gene editing and control models. In particular, through technology we developed ourselves, we revealed that ribosome stalling occurs when toxic proteins are translated.”

Professor Lim also said, "In motor neurons differentiated from induced pluripotent stem cells of Lou Gehrig's disease patients, major genes involved in protein translation quality control, such as ZNF598, are abnormally expressed," adding, "As a result, we confirmed that protein translation quality control does not function properly." He added, "The functional analysis and control technology of protein translation quality control will provide a new breakthrough in the prediction, diagnosis, and development of treatment technologies for related diseases such as Lou Gehrig's disease and frontotemporal dementia."

The research results were published in the online edition of the life sciences journal ‘Nucleic Acid Research’ on the 22nd.

This content was produced with the assistance of AI translation services.