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         Phenylketonuria-pku Disorder:     more detail
  1. 21st Century Ultimate Medical Guide to Phenylketonuria (PKU) - Authoritative Clinical Information for Physicians and Patients (Two CD-ROM Set) by PM Medical Health News, 2009-06-05
  2. Phenylketonuria (PKU) Toolkit - Comprehensive Medical Encyclopedia with Treatment Options, Clinical Data, and Practical Information (Two CD-ROM Set) by U.S. Government, 2009-06-05
  3. 2009 Empowered Patient's Complete Reference to Phenylketonuria (PKU) - Diagnosis, Treatment Options, Prognosis (Two CD-ROM Set) by PM Medical Health News, 2009-06-05
  4. Management of PKU
  5. Treatment programs for PKU and selected other metabolic diseases in the United States: A survey (DHHS publication) by Virginia E Schuett, 1983
  6. A journey into the world of PKU by Kenneth W Wessel, 1991
  7. S.B. 3018: Mandatory screening tests for PKU (University of Washington. School of Law. Student papers) by David Ward, 1974

1. Chapter 14
Phenylketonuria (PKU) Disorder occurs in one in 15,000 births. Individuals accumulate amino acid phenylalanine and its derivative phenylpyruvate in the blood to toxic levels.
http://teacher.edmonds.wednet.edu/lhs/mcaesar/documents/ch._14_2009_mendelan_gen
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2. Tags Starting With G
Where Knowledge Rules
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3. PKU | Phenylketonuria | PKU Disorder
Information on Phenylketonuria, its symptoms and proposed treatments. Learn everything about the PKU disorder and how you can identify the symptoms of PKU in kids.
http://www.birth.com.au/Newborn-screening-blood-test/Phenylketonuria.aspx
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Phenylketonuria
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Reference
Phenylketonuria
Phenylketonuria (or PKU) is a rare inherited disorder where the baby has an absence, or low level, of a liver enzyme called phenylalanine hydroxylase. This enzyme is needed to break down part of the protein in the diet called phenylalanine, an amino acid (one of many amino acids that make up protein).

4. BBlue Diaper Syndrome And Phenylketonuria Maisa Haddad
One of interest and that was not limited on information was the phenylketonuria (PKU) disorder. This was also caused by inborn error's of amino acid metabolism.
http://www-personal.umd.umich.edu/~jcthomas/JCTHOMAS/1997 Case Studies/M Haddad.
Blue Diaper Syndrome and Phenylketonuria
Maisa Haddad
Introduction
Blue diaper syndrome is an inborn error of amino acid metabolism. The amino acid tryptophan is malabsorped in the intestinal lining of newborn infants. The syndrome is known to be an autosomal recessive trait. It is believed that a mutation in the tryptophan transport protein causes a baby's diaper to turn blue once the urine touches the diaper. This was caused by the bacteria that acted on urine precursor's to produce a compound that turned indigo blue upon contact with air.Unfortenutely there was limited information on blue diaper syndrome , but there were other disorders that were caused by inborn error's of amino acid metabolism.
One of interest and that was not limited on information was the phenylketonuria (PKU) disorder. This was also caused by inborn error's of amino acid metabolism. It is caused by the deficiency of the hepatic enzyme phenylalanine hydroxylase (PAH).The amino acid phenylalanine is malabsorped because there is low concentration's of the PAH enzyme. Since there are low concentration of this enzyme , the amino acid is not being absorbed like it normally should. Therefore, there is an accumulation of the phenylalanine in the serum, which causes mental retardation in newborns.
Examples and Experiments
The difference with PKU is that there is lots of information on the disorder and actual experiments that have been done. An example is the RFLP hapotype analysis of the PAH locus in a German PKU family . A novel MspI site in one of the mutant alles was observed. The site was caused by a T to C transition in exon 9 of the gene and resulted in the substitution of leucine by proline at residue 311 of the protein (Konecki 2882). Mutagenesis and expression analyses have verified that the amino acid subsitution caused the lack of accumulation of the corresponding protein and the neucleotide substitution must represent a novel mutation in the PAH gene that causes PKU(konecki 2883). DNA sequencing of a fragment of the normal and mutant human PAH showed the T to C transition within the exon 9 gene.

5. Subject 503
Phenylketonuria (PKU) Disorder Credit 7. Business and Incentive Tax Credits Subject 506 There are several personal tax credits. These credits are figured on Lines 7A through 7D
http://www.dfa.arkansas.gov/offices/incomeTax/individual/Documents/503-TaxCredit

6. Link Between Aspartame And Formaldehyde Scaremongering By Soil And Health Spokes
Aspartame is safe for everybody, apart from those few people with the rare metabolic phenylketonuria (PKU) disorder – and aspartame must be labelled to assist them control their
http://www.nzfsa.govt.nz/publications/media-releases/2007/aspartame-and-formalde
Search Advanced Search You are here : Consumers Industry Science Verification Agency ...
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Link between aspartame and formaldehyde scaremongering by Soil and Health spokesperson
21 August 2007
NZFSA scientists say the metabolism of aspartame in the human body is well-known.
In fact, there are other foodstuffs that we ingest that supply as much and sometimes even more methanol, eg, citrus fruits and juices, and tomatoes or tomato juice. Thus, in the final analysis this methanol is the same as from other sources of food and in the quantities consumed from aspartame, it is readily and naturally metabolized via the one-carbon biochemical cycle to entirely innocuous and natural body components.
Ends For further comment contact: John Reeve, Principal Advisor (Toxicology): phone (029) 894 2533
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: Gary Bowering, Manager (Communications): phone
All information on this website is subject to a Contact for enquiries New Zealand Food Safety Authority
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NEW ZEALAND Phone: +64 4 894 2500 Fax: +64 4 894 2501 Contact NZFSA about this page Home Privacy Security ... newzealand.govt.nz

7. NZ Officials Dismiss Aspartame-formaldehyde Link
Reeve said that worldwide science was uncontested that aspartame is safe for everybody, apart from those few people with the rare metabolic phenylketonuria (PKU) disorder.
http://www.ap-foodtechnology.com/Formulation/NZ-officials-dismiss-aspartame-form

8. Linking Aspartame And Formaldehyde Scaremongering | Scoop News
Aspartame is safe for everybody, apart from those few people with the rare metabolic phenylketonuria (PKU) disorder – and aspartame must be labelled to assist them control their
http://www.scoop.co.nz/stories/SC0708/S00054.htm

9. Chapter 12
Phenylketonuria (PKU) – disorder in which the body cannot metabolize the amino acid phenylaline; Can cause brain damage
http://www.ncusd203.org/central/html/what/science/hastings/bio_chp_12.ppt
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10. An Interesting Introduction To Psychology - Initial Lifespan Development
A person with Phenylketonuria (PKU) disorder lacks an enzyme necessary for the digestion of the phenalalanine amino acid, which remains toxic to the brain in its undigested form
http://ezinearticles.com/?An-Interesting-Introduction-to-Psychology---Initial-Li

11. NZ Officials Dismiss Aspartame-formaldehyde Link
Reeve said that worldwide science was uncontested that aspartame is safe for everybody, apart from those few people with the rare metabolic phenylketonuria (PKU) disorder.
http://www.foodnavigator.com/Financial-Industry/NZ-officials-dismiss-aspartame-f

12. Link Between Aspartame And Formaldehyde Scaremongering By Soil & Health Spokespe
Aspartame is safe for everybody, apart from those few people with the rare metabolic phenylketonuria (PKU) disorder and aspartame must be labelled to assist them to control their
http://www.foodprocessing.com.au/news/12289-Link-between-aspartame-and-formaldeh

13. Pregnancy Glossary - Brand New Dad
PHENYLKETONURIA (PKU) disorder that is present when an infant does not have an enzyme necessary for the metabolism of essential proteins; a blood test is performed on infants in all
http://www.brandnewdad.com/reference/glossary.asp?letter=p

14. NZ Officials Dismiss Aspartame-formaldehyde Link
Reeve said that worldwide science was uncontested that aspartame is safe for everybody, apart from those few people with the rare metabolic phenylketonuria (PKU) disorder.
http://www.foodnavigator-usa.com/Product-Categories/Sweeteners-intense-bulk-poly

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