– About essential amino acids: Essential amino acids are amino acids that cannot be produced by the body and must be obtained through diet. An imbalance in essential amino acids could result in catabolism, or breakdown of proteins, even if overall protein needs are met. Therefore, protein synthesis is driven by essential amino acid content.
– LC-FAOD and essential amino acids: The goal of LC-FAOD management is to avoid catabolism. Thus, it is primordial to ensure that essential amino acid needs are met.
– Foods high in essential amino acids: The best sources of essential amino acids are found in animal proteins, and the highest source of essential amino acids, specifically BCAAs (branched-chain amino acids: leucine, isoleucine and valine), is whey protein. Leucine has been shown to stimulate muscle protein synthesis via mTOR signaling (1). It should be noted that essential amino acid intake in vegetarians is usually low.
– Benefit 1 – Whey protein is a complete source of protein: Another benefit of whey protein is that it constitutes a complete source of protein, meaning that it contains all 9 essential amino acids.
– Benefit 2 – Whey protein is an anaplerotic substrate (2): Whey protein is an anaplerotic substrate, meaning that it replenishes the pool of citric-acid cycle intermediates, which are usually low in LC-FAOD patients because there is little fat oxidation, which is the main provider of those intermediates. Citric cycle intermediates allow for gluconeogenesis, or the synthesis of glucose from noncarbohydrate sources (amino acids/fats), when glucose is insufficient.
– Valine oxidation in particular, which is high in whey protein, produces propionyl-coA, which is converted into succinyl-CoA.
– Benefit 3- Whey protein is ketogenic (2): The ketoacids in whey protein may provide additional substrate for the liver to produce ketones independent of fatty acid oxidation. Ketones can be used by the brain for energy.
– Benefit 4 – Beneprotein is quickly absorbed and oxidized: Beneprotein is a pharmaceutical-grade protein powder composed of whey protein and can be used as a protein supplement in LC-FAODs. Not only is it considered as a complete protein source, but it also contains more free amino acids than protein in whole foods (such as meat, eggs), which improves amino acid absorption and oxidation (3). This can be beneficial especially around exercise, where a protein that is more quickly absorbed can improve muscle synthesis. Furthermore, this powder mixes into various hot or cold foods and beverages (4).
Benefit 5 – Beneprotein can help to maintain lean body mass and lower liver lipid deposition: In a study, patients with LC-FAOD consuming a high-protein diet (composed of about 10% calories from MCT, 10% from LCT, 25% from protein (and where 25% of proteins were from beneprotein), the remaining beneprotein supplementation (which constituted about 25% of calories from protein), had a lower liver lipid deposition, preserved lean body mass, and maintained metabolic control, in comparison to the high-carbohydrate group that had higher liver lipid deposition and lower lean body mass (5).
Dosage:
Please consult your nutritionist for specific recommendations.
An article recommends 20-25g of whey protein/day (5), which corresponds to about 4 scoops/day of beneprotein (6g/scoop * 4 scoops = 24g).
Beneprotein could be supplemented to a whole food protein diet. Optimally, it should be distributed in equal doses throughout the day, for example by taking 1 scoop at every meal (6). It could also be taken right after exercise to maximize muscle synthesis.
Beneprotein can be purchased here: https://www.nestlemedicalhub.com/products/beneprotein
References:
(2) https://pubmed.ncbi.nlm.nih.gov/31295363/
(3) https://www.journalofdairyscience.org/article/S0022-0302(73)85322-6/pdf
(4) https://www.nestlemedicalhub.com/products/beneprotein
(5) https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8365340/
(6) Book ‘Nutrition management of inherited metabolic diseases’ Lessons from Metabolic University by LE Bernstein
