Familial glucocorticoid deficiency

Congenital adrenal hyperplasia ° Familial glucocorticoid deficiency and hypoplasia... 

Metherell LA, Naville D, Halaby G et al (2000) Nonclassic lipoid congenital adrenal hyperplasia masquerading as familial glucocorticoid deficiency. J Clin Endocrinol Metab 94 3865-3871... 

In rats fed a diet adequate in vitamin Bg, the fraction of total pyridoxal phosphate found in the nuclei of liver cells was 21%, and this increased to 39% in rats fed a vitamin Bg-deflcient diet, indicating a conservation of the vitamin in the nuclear compartment during deficiency. Pyridoxal phosphate in the cell nucleus is protein bound, and this protein has an apparent molecular mass of 50 to 55 kDa. Cells grown in the presence of 5 mM pyridoxine have a decreased glucocorticoid-dependent induction of enzymes such as tyrosine aminotransferase. Vitamin Bg regulates transcriptional activation of human glucocorticoid receptors in the HeLa cells. The modulatory role in transcription is not restricted to the glucocorticoid receptor but extends to other members of the steroid hormone super family. The intracellular concentration of PLP could have a profound influence on steroid hormone-induced... 

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