Description) The focus of this revised application is to determine if a correlation exists between myelination and neurotransmitter production in phenylketonuria. The investigator's preliminary data show that myelination, demyelination, and remyelination take place in PKU mice as they are cycled on rounds of low Phenylalanine diet and regular diet. Since blood Phe levels change rapidly compared to changes in myelination, she proposed to examine the relationship between gain and loss of myelin and neurotransmitter production. The investigator will employ histochemical, immunohistochemical, molecular, and biochemical techniques to better understand the pathophysiology of PKU, and ultimately lead to the development of additional therapies for PKU.

Agency
National Institute of Health (NIH)
Institute
Eunice Kennedy Shriver National Institute of Child Health & Human Development (NICHD)
Type
Small Research Grants (R03)
Project #
5R03HD038522-02
Application #
6402707
Study Section
Pediatrics Subcommittee (CHHD)
Program Officer
Oster-Granite, Mary Lou
Project Start
2000-08-01
Project End
2003-07-31
Budget Start
2001-08-01
Budget End
2003-07-31
Support Year
2
Fiscal Year
2001
Total Cost
$85,000
Indirect Cost
Name
Children's Hospital of Philadelphia
Department
Type
DUNS #
073757627
City
Philadelphia
State
PA
Country
United States
Zip Code
19104
Joseph, Bernadin; Dyer, Charissa A (2003) Relationship between myelin production and dopamine synthesis in the PKU mouse brain. J Neurochem 86:615-26