This subproject is one of many research subprojects utilizing theresources provided by a Center grant funded by NIH/NCRR. The subproject andinvestigator (PI) may have received primary funding from another NIH source,and thus could be represented in other CRISP entries. The institution listed isfor the Center, which is not necessarily the institution for the investigator.The overall goal of this proposal is to achieve a deeper understanding of in vivo nitrogen metabolism in human, with specific foci on interactions between essential amino acid metabolism and urea synthesis and on diseases affecting the urea cycle. Our past work has indicated that in comparison with normal individuals, patients with genetic defects in urea synthesis may exhibit a partial block in their ability to utilize gluatmine as a urea nitrogen source. As a consequence they rely more on enterally generated nitrogen as a source of urea. In addition we and others have also shown that a common therapy for such patients, (i.e., the ingestions ofeither phenylbutyrate or phenylacetate, often in combination with benzoate) diverts nitrogen from the urea synthesis to alternative routes of excretion (and hence achieves its therapeutic objective). However,our studies suggest that this therapeutic modality has a substantial impact on branched chain amino acid metabolism (BCAA). In fact, this meabolic disruption leads to a marked fall in BCAA concentrations in spite of adequate levels of total protein intake. One predicted outcome of the effect of these druge, is to inhibit body protein synthesis. Uniquely,this clinical scenario may provide an opportunity to selectively evaluate the role of branch chain amino acids in regulating global protein synthesis. Accordingly, we propsoe experiments in normal subjects and in patients suffering from partial and/or severe defects in urea synthesis to test these hypotheses. These findings should directly impact on our nutritional management of these and other disorders in which restriction of protein intake is a main stay of therapy.

Agency
National Institute of Health (NIH)
Institute
National Center for Research Resources (NCRR)
Type
General Clinical Research Centers Program (M01)
Project #
2M01RR000188-43
Application #
7605838
Study Section
National Center for Research Resources Initial Review Group (RIRG)
Project Start
2007-02-15
Project End
2007-11-30
Budget Start
2007-02-15
Budget End
2007-11-30
Support Year
43
Fiscal Year
2007
Total Cost
$49,207
Indirect Cost
Name
Baylor College of Medicine
Department
Pediatrics
Type
Schools of Medicine
DUNS #
051113330
City
Houston
State
TX
Country
United States
Zip Code
77030
Hunsaker, Sanita L; Garland, Beth H; Rofey, Dana et al. (2018) A Multisite 2-Year Follow Up of Psychopathology Prevalence, Predictors, and Correlates Among Adolescents Who Did or Did Not Undergo Weight Loss Surgery. J Adolesc Health 63:142-150
Lanzieri, Tatiana M; Chung, Winnie; Leung, Jessica et al. (2018) Hearing Trajectory in Children with Congenital Cytomegalovirus Infection. Otolaryngol Head Neck Surg 158:736-744
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Oh, Sam S; Du, Randal; Zeiger, Andrew M et al. (2017) Breastfeeding associated with higher lung function in African American youths with asthma. J Asthma 54:856-865
Bansal, N; Hampe, C S; Rodriguez, L et al. (2017) DPD epitope-specific glutamic acid decarboxylase (GAD)65 autoantibodies in children with Type 1 diabetes. Diabet Med 34:641-646
Zeller, Meg H; Washington, Gia A; Mitchell, James E et al. (2017) Alcohol use risk in adolescents 2 years after bariatric surgery. Surg Obes Relat Dis 13:85-94

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