A dangerous buildup of phenylalanine can develop when a person with PKU eats protein-rich foods, such as milk, cheese, nuts or meat, and even grains such as bread and pasta, or eats aspartame, an artificial sweetener. Info With our food browser, you can sort thousands of different foods and recipes by phenylalanine, or by dozens of other nutrients. Phenylalanine is an amino acid used to make proteins and brain chemicals including dopamine, adrenaline and thyroid hormones. It’s generally considered safe, but people with phenylketonuria (PKU) may experience dangerous side effects. Accessed Nov. 14, 2018. Your body forms thousands of different types of protein — all crucial to your health. Electronic Code of Federal Regulations. Pregnant women are advised to avoid it, as are those with the disorder PKU who are genetically unable to properly process phenylalanine and usually are directed to eat a low-protein diet throughout their lives. Alternatively, you can buy frozen meals that are organic to cut down on preservatives, additives, and artificial sweeteners. The only treatment for PKU is to avoid eating foods that contain phenylalanine. They will also be labelled 'Contains a source of phenylalanine'. However, do not take more. Phenylketonuria (fen-ul-key-toe-NU-ree-uh), also called PKU, is a rare inherited disorder that causes an amino acid called phenylalanine to build up in the body. meat, fish, milk and eggs) and cereal protein sources (e.g. https://rarediseases.info.nih.gov/diseases/7383/phenylketonuria. Eating a variety of protein-rich foods throughout the day will provide you with all the amino acids your body needs, including phenylalanine. Additionally, phenylalanine is found in aspartame, the sweetener found in most diet soft drinks and sugar-free candies/gum - hence the need for the warning on the labels of these products. All rights reserved. This article compares animal and plant proteins. Phenylalanine is found in most protein-containing foods, so people with PKU are advised to follow a special low-protein diet. People with PKU must also avoid food products that contain aspartame, as it's converted into phenylalanine in the body. Accessed July 3, 2018. In addition to its role in protein production, phenylalanine is used to make other important molecules in your body, several of which send signals between different parts of your body (6). Later, high protein foods must be restricted, along with certain other phenylalanine-containing foods, which includes anything containing aspartame. Does soy really affect breast cancer risk? Overview of phenylketonuria. Science says change your eating space. Phenylalanine is one of the so-called 'essential' amino acids and must be obtained by the body from food. Aspartame contains phenylalanine and therefore it must be avoided. Scientists have been able to show that carriers had higher phenylalanine levels in their urine than non-carriers. Phenylalanine can cause intellectual disabilities, brain damage, seizures and other problems in people with PKU. Phenylalanine can be converted to the amino acid tyrosine, which is then used to produce important signaling molecules. Tyrosine Vs. Phenylalanine. Phenylalanine is an amino acid found in natural protein. It's commonly sold as NutraSweet or Equal,but it's listed as aspartamein diet, lite, or sugar-free foods like soft drinks, desserts, gelatins, puddings, yogurts, etc. Phenylketonuria is a hereditary metabolic disease, usually diagnosed at birth, characterized by an inability by the body to effectively process phenylalanine. Can you get phenylalanine naturally from foods? In animal (e.g. This buildup of phenylalanine results in damage to nerve cells in the brain. Phenylalanine is an amino acid used to make proteins and brain chemicals including dopamine, adrenaline and thyroid hormones. Your body needs phenylalanine and other amino acids to make proteins. Check out these best-sellers and special offers on books and newsletters from Mayo Clinic. Milk and cheese 6. Animal vs. Plant Protein — What's the Difference? It is also present in bananas. Other studies have reported that adding phenylalanine supplements to ultraviolet (UV) light exposure may improve skin pigmentation in individuals with this condition (13, 14). Here are 9 important functions of the protein in your body. wheat flour and breakfast cereal), usually the amount of food that is calculated to provide 1 g protein will supply approximately 50 mg phenylalanine. Vaccine updates, safe care and visitor guidelines, and trusted coronavirus information, Mayo Clinic Graduate School of Biomedical Sciences, Mayo Clinic School of Continuous Professional Development, Mayo Clinic School of Graduate Medical Education, Play it safe when taking food to a loved one in the hospital, Book: Mayo Clinic Family Health Book, 5th Edition, Book: Mayo Clinic 5 Steps to Controlling High Blood Pressure, Newsletter: Mayo Clinic Health Letter — Digital Edition, 3 diet changes women over 50 should make right now, 3 key changes in the new Nutrition Facts label, Artificial sweeteners and other sugar substitutes, Autism spectrum disorder and digestive symptoms, Bad food habits at work? Babies are put on a special formula that does not contain phenylalanine. Protein is an important nutrient for optimal health, but not all protein sources are equal. Why does diet matter after bariatric surgery? Eating a variety of protein-rich foods throughout the day will provide you with all the phenylalanine you need, along with other essential amino acids. A child with a level less than 6 mg/dl does not need to be on a special diet and is not risk for mental retardation, but should avoid aspartame at all cost due to the 50% phenylalanine levels. Phenylalanine is also sold as a dietary supplement. Eggs are among the healthiest and most nutritious foods on the planet. As for phenylalanine, while it does not offer a stimulating effect, it may resolve a deficiency in tyrosine. Who Should Avoid These Substances. Phenylalanine is found in most protein-containing foods such as milk, eggs, cheese, nuts, … The treatment of PKU it’s usually dietary modification. Many people who follow a low phenylalanine (phe) food pattern eat special low protein breads and pastas. What's the difference between juicing and blending? This molecule exists in two forms or arrangements: L-phenylalanine and D-phenylalanine. Phenylalanine is an amino acid found in many foods and used by your body to produce proteins and other important molecules. Soy protein supplements can provide about 2.5 grams of phenylalanine per 200-calorie serving (5, 29). Found in many foods, this amino acid builds important neurotransmitters and proteins. Better Choices for Healthy Weight Loss The Better Choices approach predicts that foods closer to the top of this list are more filling and more nutritious per calorie than foods farther down the list, and therefore are better for healthy-weight-loss diets. These warnings are placed to help individuals avoid such foods. They should avoid high-protein foods such as: 1. Many studies suggest that nutrition plays a role in this common disorder. The choice boils down to an individual’s preference. A defective gene (genetic mutation) causes PKU, which can be mild, moderate or severe. Eggs 5. Many important proteins are found in your brain, blood, muscles, internal organs and virtually everywhere else in your body. PKU is caused by a defect in the gene that helps create the enzyme needed to break down phenylalanine.Without the enzyme necessary to process phenylalanine, a dangerous buildup can develop when a person with PKU eats foods that contain protein or eats asp… Phenylalanine is found in a variety of foods, including almonds, avocados, bananas, brown rice, cheese, corn, eggs, fish, lima beans, peanuts, pumpkin seeds, sesame seeds, and soy products. Because people with PKU are unable to metabolize phenylalanine properly, they should avoid or limit its intake from food and drink. They are nearly free of phe, allow greater freedom in food choices, and provide energy and variety in the food pattern. Special phenylalanine-free foods are available for children and teens with PKU. Your body is unable to produce enough L-phenylalanine on its own, so it’s considered an essential amino acid that must be obtained through your diet (4). Many foods, including soy products, eggs, seafood and meats, contain phenylalanine. Ingesting minute amounts (calculated by a nutritionist) of regular formula or breast milk can help meet minimum phenylalanine needs. Because of this, use products with aspartame cautiously if you: If you aren't sure if phenylalanine or aspartame is a concern for you, talk to your doctor. Try replacing some food products with foods that are organic. Since phenylalanine is used to make these molecules in your body, it has been studied as a potential treatment for certain conditions, including depression (9). Foods highest in Phenylalanine (based on levels per 200-Calorie serving) Baked Products (1) Vegetables and Vegetable Products (32) Nut and Seed Products (6) BCAA stands for branched-chain amino acids. Poultry (chicken and turkey) 3. Because phenylalanine is an amino acid, it's not surprising that high-protein foods are the primary foods to avoid if you have PKU. © 1998-2020 Mayo Foundation for Medical Education and Research (MFMER). It has been studied for its effects on depression, pain and skin disorders. The role of diet and exercise in preventing Alzheimer's disease. Scientists have been able to show that carriers had higher phenylalanine levels in … Inheritance The artificial sweetener aspartame (Equal, NutraSweet), which is added to many medications, diet foods and diet sodas, contains phenylalanine. Accessed July 3, 2018. https://www.fda.gov/Food/IngredientsPackagingLabeling/FoodAdditivesIngredients/ucm397725.htm#Aspartame. This warning helps people with PKU avoid products that are a source of phenylalanine. (109% RDI) … Phenylalanine‐free or low‐phenylalanine proteins and peptides in food provide not only the essential AAs but also the functional properties needed for making variety in foods (van Calcar and Ney 2012). In a person with PKU, this defective gene causes a lack of or deficiency of the enzyme that's needed to process phenylalanine, an amino acid. The artificial sweetener aspartame (Equal, NutraSweet), which is added to many medications, diet foods and diet sodas, contains phenylalanine. For otherwise healthy individuals, phenylalanine is still essential, and can easily be gained from eating foods high in phenylalanine. Aspartame. 100 grams of Pigeon pea contains 0% of the Phenylalanine… Here are important facts about these essential amino acids. Phenylalanine is one of … These dangerously high concentrations can cause brain damage and intellectual disability, as well as problems with the transport of other amino acids to the brain (7, 28). 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