NDLI: Cytochrome b $_{5}$ null mouse: a new model for studying inherited skin disorders and the role of unsaturated fatty acids in normal homeostasis

Content Provider	Springer Nature Link
Author	Finn, Robert D. McLaughlin, Lesley A. Hughes, Catherine Song, Chengli Henderson, Colin J. Roland Wolf, C.
Copyright Year	2010
Abstract	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.
Starting Page	491
Ending Page	502
Page Count	12
File Format	PDF
ISSN	09628819
Journal	Transgenic Research
Volume Number	20
Issue Number	3
e-ISSN	15739368
Language	English
Publisher	Springer Netherlands
Publisher Date	2010-07-30
Publisher Place	Dordrecht
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	Cytochrome b $_{5}$ Ichthyosis Methaemoglobinemia Nutrition Skin Unsaturated fatty acids Animal Genetics and Genomics Plant Sciences Molecular Medicine Plant Genetics & Genomics Human Genetics
Content Type	Text
Resource Type	Article
Subject	Genetics Animal Science and Zoology Biotechnology Agronomy and Crop Science

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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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Cytochrome b $_{5}$ null mouse: a new model for studying inherited skin disorders and the role of unsaturated fatty acids in normal homeostasis

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Cytochrome b $_{5}$ null mouse: a new model for studying inherited skin disorders and the role of unsaturated fatty acids in normal homeostasis