Steroid Deficiency Syndromes in Mice with Targeted Disruption of Cyp11a1
| Autor: | Meng Chun Hu, Nai Chi Hsu, Hsueh-Ping Chu, Bon Chu Chung, Chi-Kuang Leo Wang, Noomen Ben El Hadj, Chin I. Pai |
|---|---|
| Rok vydání: | 2002 |
| Předmět: |
Male
endocrine system medicine.medical_specialty Steroid 21-Hydroxylase medicine.medical_treatment Gene Expression Genitalia Male Biology Steroid Electrolytes Mice Endocrinology Cytochrome P-450 Enzyme System Internal medicine Adrenal Glands medicine Animals Feminization Cholesterol Side-Chain Cleavage Enzyme Molecular Biology Mice Knockout Sexual differentiation Cholesterol side-chain cleavage enzyme Vas deferens Gene targeting Syndrome General Medicine Lipid Metabolism Epididymis medicine.anatomical_structure Gene Targeting Steroids Glucocorticoid medicine.drug |
| Zdroj: | Molecular Endocrinology. 16:1943-1950 |
| ISSN: | 1944-9917 0888-8809 |
| Popis: | Steroid deficiencies are diseases affecting salt levels, sugar levels, and sexual differentiation. To study steroid deficiency in more detail, we used a gene-targeting technique to insert a neo gene into the first exon to disrupt Cyp11a1, the first gene in steroid biosynthetic pathways. Cyp11a1 null mice do not synthesize steroids. They die shortly after birth, but can be rescued by steroid injection. Due to the lack of feedback inhibition by glucocorticoid, their circulating ACTH levels are exceedingly high; this results in ectopic Cyp21 gene expression in the testis. Male Cyp11a1 null mice are feminized with female external genitalia and underdeveloped male accessory sex organs. Their testis, epididymis, and vas deferens are present, but undersized. In addition, their adrenals and gonads accumulate excessive amounts of lipid. The lack of steroid production, abnormal gene expression, and aberrant reproductive organ development resemble various steroid deficiency syndromes, making these mice good models for studies of steroid function and regulation. |
| Databáze: | OpenAIRE |
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