These Hsd3b7-/- mice lack exons 1-6 of the hydroxy-delta-5-steroid dehydrogenase, 3 beta- and steroid delta-isomerase 7 (Hsd3b7) gene, abolishing gene expression. Hsd3b7 encodes an oxidoreductase which catalyzes two reactions required for the synthesis of bile acids from cholesterol in the small intestines and liver. Mutations of Hsd3b7 have been implicated in recessive forms of neonatal liver failure. Heterozygotes are viable, fertile, and normal in size. Homozygotes exhibit reduced growth and decreased survival; 45% of mice die during the first 4 days of life, and another 45% die between 8 and 18 days of age when maintained on a normal chow diet. These mice exhibit decreased intestinal cholesterol absorption, serum triglyceride levels, and cholesterol biosynthesis in the kidney. They also exhibit slight increases in fecal bile acid and steroid secretion, and increased cholesterol biosynthesis in the liver and small intestine. Hepatic fatty acid biosynthesis and triglyceride levels are also increased. These homozygous mice have oily fur and scaly skin. When mice are maintained on vitamin and bile acid supplements for the first 28 days of life, these mice appear normal and have decreased serum triglyceride levels and increased hepatic triglyceride levels when compared to wildtype controls.

These Hsd3b7 knockout mice may be useful for studying cholesterol and bile acid biosynthesis and homeostasis.

Typically mice are recovered in 12-16 weeks. Contact Customer Service to place an order or for more information.

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