![]() ![]() Histological analyses showed alterations in several structures in white and gray matters in 35 day-old PDC-deficient females. The oxidation of glucose to CO 2 and incorporation of glucose-carbon into fatty acids were reduced in brain slices from 15 day-old PDC-deficient females. Immunohistochemical analysis of several brain regions showed that approximately 40% of cells were PDH −. PDC activity was reduced in the brain and other tissues in female progeny compared to age-matched control females. ![]() RESULTS: Male embryos died prenatally but heterozygous females were born. ![]() METHODS: In the present study we investigated a murine model of systemic PDC deficiency by interrupting the X-linked Pdha1 gene encoding the α subunit of PDH to study its role on brain development and behavioral studies. Systematic analyses of development of affected brain structures and the cellular processes responsible for their impairment have not been performed due to the lack of an animal model for PDC deficiency. Pyruvate dehydrogenase (PDH) complex (PDC) deficiency is an inborn error of pyruvate metabolism causing a variety of neurologic manifestations. ![]()
0 Comments
Leave a Reply. |
AuthorWrite something about yourself. No need to be fancy, just an overview. ArchivesCategories |