between yeast and people

Can yeast - single-celled microorganisms - teach us about a neurological disease that attacks humans?

Prof. Geoffrey Gerst and Rachel Kama. Transport routes
Can yeast - single-celled microorganisms - teach us about a neurological disease that attacks humans? It turns out that, despite the billions of years of evolution that separate us from the yeast, there are quite a few basic cellular functions that operate in a similar way in yeast cells and human cells. Here is another reason for humility, that lost and so rare quality that can lead to the correction of the world. Prof. Jeffrey Gerst, from the Department of Molecular Genetics at the Weizmann Institute of Science, recently showed that yeast cells can be used as a reliable model for studying a rare disease that attacks humans. This work, which was recently described in an article published in the scientific journal Molecular and Cellular Biology, was carried out together with Rachel Kama and research student Micah Robinson.
Batten disease is a neurodegenerative disease that occurs in one in 50,000 children. It is caused when both copies of the gene called BTN2 are damaged. The disease manifests itself in mental retardation, loss of vision and motor skills, and eventually causes death. The cells living in the bodies of patients with the disease fail to break down and eliminate certain substances that have finished their role in the cell, which leads to the accumulation of these substances in the cells. In healthy cells (in which the gene is not damaged) the process of breaking down and eliminating these substances takes place in an intracellular organelle called a lysosome. This group of diseases (lysosomal storage diseases) also includes diseases such as Gaucher, Tay-Sachs, and
which appear in older humans and is called by the general name Neuronal Ceroid Lipofuscinoses. Most storage diseases currently have no cures.
Prof. Gerst and the members of his research group accidentally encountered the lysosomal storage diseases that exist in humans in their research. cellular
Yeast is used as a model for studying basic functions carried out in all living cells, including ways of transporting proteins and other products that are moved from place to place inside the cell.
Movement in the intracellular transport pathways flows according to a complex set of rules, which scientists around the world have only recently begun to decipher. This system includes transport centers - the Golgi organelles - from which vesicles are secreted that serve as transport containers. The lysosomes (whose dysfunction causes lysosomal storage diseases), are formed in part from the Golgi organelles. The lysosomes engulf proteins whose function is over, and break them down. But some proteins do not reach the lysosomes, and are returned to the Golgi organelles for recycling.
The scientists found that the Batten disease-related gene, BTN2, in its version found in yeast cells, works together with another gene, which codes for a protein that plays an important role in the intracellular transport pathway. The BTN2 gene has been studied in the past by various scientists, but its role remains unclear.
By deleting the BTN2 gene from the genetic load of the yeast cells, the members of the research group were able to simulate human cells in the bodies of patients with the disease (in which both copies of the BTN2 gene do not function properly), and find out with which proteins the protein produced according to this gene works under normal conditions (without disease) , and where in the intracellular transport system does the disruption occur that ultimately causes the storage diseases. They found that the lack (or dysfunction) of the BTN2 gene disrupts the ability of the intracellular transport pathways to transport a protein called Yif1. Normally, the Yif1 protein molecules are transported back to the Golgi organelles for recycling. However, in the disease model cells (which lack the Btn2 protein) this path is blocked, so they are directed in the wrong direction - into the lysosome, where they are broken down instead of being recycled. The unwanted products of the incorrect decomposition accumulate in the lysosome - just as happens in the cells of people suffering from Batten's disease.
The institute's scientists revealed the exact stage at which the Yif1 transport pathway begins to malfunction as a result of the lack (or lack of function) of the BTN2 gene. Prof. Gerst: "We saw that the mechanism leading to the development of Batten's disease is largely similar in yeast and in humans. It turns out that these tiny cells serve as an excellent model for understanding the development processes of various diseases in humans. Such an understanding is an essential step on the way to developing treatment methods for these diseases."