Researchers Discover New Mechanism of Neurodegeneration

Saturday, November 23, 2019 - 14:27

Scientists from the VIB-UAntwerp Center for Molecular Neurology and the Scripps Research Institute have discovered that a class of cytoplasmic enzymes called tRNA synthetases can cause CMT by interfering with the gene transcription in the nucleus.

According to the Technology Network reports, scientists, released their findings in the leading journal Nature Communications, demonstrated that a class of cytoplasmic enzymes called tRNA synthetases can cause CMT by interfering with the gene transcription in the nucleus.

Charcot-Marie-Tooth disease (CMT) is a condition that affects the peripheral nervous system. It leads to progressive muscle weakness and loss of sensation in the lower and - later on - upper limbs.

Researchers gained a better understanding of the CMT disease mechanisms that can be applicable for other neurogenerative disorders too.

The scientists found that in the cell core - the nucleus - of human cell cultures and Drosophila models something went wrong. A major process that happens in the nucleus is the transcription of genetic information encrypted in DNA into RNA molecules, which are then exported in the cytoplasm of the cell and translated into proteins there.

The researchers uncovered that an important group of molecules known as aminoacyl-tRNA synthetases - which help in translating RNA into proteins - can also interfere with the transcription of DNA into RNA. This interference was found to be at the core of CMT disease in both fly and cellular models.

Prof. Albena Jordanova explains: "The fundamental message from our work is that components of the translational machinery can function as transcriptional regulators in the nucleus. We demonstrate for the first time that their nuclear role has pathological implications and can cause a neurodegenerative disease. This breaks the current dogmas on the known function of aminoacyl-tRNA synthetases and changes our view on how to study their associated diseases."

Dr. Sven Bervoets, first author of the study, explains: "Pharmaceutical inhibition of the tRNA synthetase entry into the nucleus prevented the onset of disease symptoms in our CMT Drosophila model, which could have great implications for CMT patients."

Opinions


Popular News

Latest News