Wednesday, January 2, 2013

Future Prospects of research to tackle Phenylketonuria



One promising therapy involves oral administration of recombinant phenylalanine lyase (PAL), an enzyme that degrades phenylalanine in the intestinal tract into trans-cinnamic acid that can be broken down in the liver of people with PAH deficiency. Animal studies and limited human studies have suggested that PAL has the potential to make the PKU diet less restrictive for humans. It has been tested using intraperitoneal and subcutaneous routes. The utility and feasibility of enzyme replacement using PAL continue to be evaluated, though less progress has been made. This avenue is still open to research and lots of trails are underway currently. The key to an effective treatment method would be to have sufficient bioavailability, minimal side effects and consistent effectiveness.

Research on foods and supplements also shows promise for improvong the lives of those affectcted by PKU. Glycomacropeptide (GMP) is a whey protein and the only protein free of Phe; researchers have been able to to successsfully create foods out of GMP that were met with positive reviews by people with PKU who tried them. This GMP food may be made available to the common public commercially in time to come.

Reference

  • Patricia Jackson Allen, Judith A Vessey,  2010. Primary care of the Child with a Chronic Condition . 5th ed. United States of America: Mosby Elsevier.



2 comments:

  1. Using of GMP in foods, will in have any adverse effects on the consumer? Is it safe?

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  2. hi dskt!

    In most cases it does not have any adverse effects on consumers.

    "There are no known adverse effects of glycomacropeptide." - http://www.fitnessforworld.com/supplement/glycomacropeptide.htm

    The only possibility of side effects is when, people with lactose intolerance consume it. Whey protein is derived from milk and hence people with lactose intolerance may experience additional side effects or some may develop allergic reactions. But again this is only when the person has an allergy to dairy proteins.

    - Mukesh and Zhi Zheng

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