Cytochrome P450s in the synthesis of @entity165 and @entity3645 --from @entity19 models to XXXX diseases.

@entity1 @entity38296 @entity52501 @entity17971 @entity451 @entity61121 @entity19 @entity30050 @entity6329 @entity73 @entity33349 @entity6742 @entity4740 @entity6741 @entity17283 @entity51330 @entity3645 @entity204 @entity165 @entity19580
The present review describes the transgenic @entity19 models that have been designed to evaluate the functions of the cytochrome P450s involved in @entity165 and @entity6741 synthesis, as well as their link with disease. The knockout of cholesterogenic @entity51330 is embrionally lethal, with symptoms of @entity17971 occurring in @entity19 , whereas the evidence for this association is conflicting in @entity1 . Disruption of @entity38296 from classic @entity6741 synthesis in @entity19 leads to either @entity204 or a milder phenotype with elevated serum @entity165 . The latter is similar to the case in @entity1 , where @entity30050 mutations associate with high plasma low-density lipoprotein and hepatic @entity165 content, as well as deficient @entity6741 excretion. Disruption of @entity6329 from an alternative @entity6741 pathway results in the absence of @entity6742 and a reduced absorption of dietary lipids; however, the @entity1 @entity6329 polymorphism fails to explain differences in @entity6741 composition. Unexpectedly, apparently normal @entity52501 (-/-) @entity19 still synthesize @entity3645 that originate from the compensatory pathway. In @entity1 , @entity17283 mutations cause @entity4740 , suggesting that only @entity19 can compensate for the loss of alternative @entity6741 synthesis. In line with this, @entity61121 knockouts are also apparently normal, whereas @entity1 @entity61121 mutations lead to a congenital @entity6741 synthesis defect in @entity1 or @entity451 in adults. @entity19 knockouts of the brain-specific @entity19580 have reduced brain @entity165 excretion, whereas, in @entity1 , @entity33349 polymorphisms associate with @entity73 . At present, cytochrome P450 family 39 is poorly characterized. Despite important physiological differences between @entity1 and @entity19 , @entity19 models prove to be an invaluable tool for understanding the multifactorial facets of @entity165 and @entity6741 -related disorders.
