mTOR and Transcription Factor EB (TFEB)

Questions on how we could avoid using the GSD II pathways and how to best clear out a lysosome is what led us to mTOR and EB (TFEB).   The papers below will help you and your doctors connect the dots between the ketogenic diet, mTOR and transcription factor EB (TFEB).  All three papers are free to read (Click to open).

  1. The ketogenic diet inhibits the mammalian target of the rapamycin (mTOR) pathway.

  2. mTOR Signaling in Growth, Metabolism, and Disease.

  3. TFEB at a glance.

From the third study above, TFEB at a glance: The following graphic, Mechanism of TFEB activation, shows one side is red and has an up arrow next to the word mTOR and a down arrow next to the word TFEB. The other side is green and shows a down arrow next to mTOR and an up arrow next to TFEB. We are now testing the idea that a properly formulated ketogenic diet has the ability to shift a person’s metabolism over to the green side of this graphic more often. This allows TFEB to move down to the nucleus and express itself.

This next graphic is also from the paper TFEB at a glance, and it shows the processes that happen when TFEB moves to the nucleus and is expressed.

By using a properly formulated Ketogenic Diet, we think it’s possible to overexpress TFEB and limit lysosomal-glycogen buildup in the body. PWF is hypothesizing that the overexpression of TFEB is a treatment that would improve the standard of care for Pompe Disease.