Cytochrome b5 null mouse: a new model for studying inherited skin disorders and the role of unsaturated fatty acids in normal homeostasis.
Cytochrome b5 null mouse: a new model for studying inherited skin disorders and the role of unsaturated fatty acids in normal homeostasis.
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DOI：
10.1007/s11248-010-9426-1
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发表时间：
2011-06
影响因子：
3
通讯作者：
Roland Wolf C
中科院分区：
文献类型：
--
作者：
Finn RD;McLaughlin LA;Hughes C;Song C;Henderson CJ;Roland Wolf C
Microsomal cytochrome b 5 is a ubiquitous, 15.2 kDa haemoprotein implicated in a number of cellular processes such as fatty acid desaturation, drug metabolism, steroid hormone biosynthesis and methaemoglobin reduction. As a consequence of these functions this protein has been considered essential for life. Most of the ascribed functions of cytochrome b 5, however, stem from in vitro studies and for this reason we have carried out a germline deletion of this enzyme. We have unexpectedly found that cytochrome b 5 null mice were viable and fertile, with pups being born at expected Mendelian ratios. However, a number of intriguing phenotypes were identified, including altered drug metabolism, methaemoglobinemia and disrupted steroid hormone homeostasis. In addition to these previously identified roles for this protein, cytochrome b 5 null mice displayed skin defects closely resembling those observed in autosomal recessive congenital ichthyosis and retardation of neonatal development, indicating that this protein, possibly as a consequence of its role in the de novo biosynthesis of unsaturated fatty acids, plays a central role in skin development and neonatal nutrition. Results from fatty acid profile analysis of several tissues suggest that cytochrome b 5 plays a role controlling saturated/unsaturated homeostasis. These data demonstrate that regional concentrations of unsaturated fatty acids are controlled by endogenous metabolic pathways and not by diet alone. The online version of this article (doi:10.1007/s11248-010-9426-1) contains supplementary material, which is available to authorized users.
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影响因子：
6.5
作者：
Attie, AD;Krauss, RM;Ntambi, JM
通讯作者：
Ntambi, JM
影响因子：
4.2
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Miyazaki, M;Man, WC;Ntambi, JM
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de Juanes, Silvia;Epp, Nikolas;Krieg, Peter
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3.6
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Bongiovanni, Kathleen D.;Depeters, Edward J.;Van Eenennaam, Alison L.
通讯作者：
Van Eenennaam, Alison L.