Cardiovascular Defects among the Progeny of Mouse Phenylketonuria Females
| Autor: | Margaret L. Kirby, McDonald Jd, Dyer Ca, Gailis L |
|---|---|
| Rok vydání: | 1997 |
| Předmět: |
Heart Defects
Congenital medicine.medical_specialty Genotype Phenylketonurias Offspring Phenylalanine Disease Biology Mice Pregnancy Internal medicine medicine Animals Humans Maternal-Fetal Exchange Fetus medicine.disease Diet Pregnancy Complications Disease Models Animal Endocrinology Pediatrics Perinatology and Child Health Blood Vessels Gestation Female |
| Zdroj: | Pediatric Research. 42:103-107 |
| ISSN: | 1530-0447 0031-3998 |
| DOI: | 10.1203/00006450-199707000-00016 |
| Popis: | In a genetic mouse model of human phenylketonuria we have examined the offspring of hyperphenylalaninemic mothers for the presence of cardiovascular defects, an important feature of the pathology of the human maternal phenylketonuria syndrome. Beginning at 14.5 d after conception (75% through gestation), a variety of cardiovascular defects became apparent among the progeny of the hyperphenylalaninemic females. These defects ranged from mild to serious and correlated with the maternal but not the fetal Pah genotype. Nearly all of the defects were vascular, however, whereas the most reported in humans so far have been cardiac. The predisposing biochemical condition in this mouse disease model seems to be the same as in the human disease; elevated maternal blood phenylalanine levels concentrated across the placental barrier to produce a teratogenic developmental environment. This model for congenital cardiovascular defects should enhance two related areas of research. 1) It should allow a more thorough investigation of the relationship between maternal diet and maternal phenylketonuria birth defects, and 2) it should provide an experimental tool to gain insight into the normal process of cardiovascular development. |
| Databáze: | OpenAIRE |
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