Homozygous whole body Cbs knockout in adult mice features minimal pathology during ageing despite severe homocysteinemia
| Autor: | D. Nakladal, S. P. H. Lambooy, S. Mišúth, D. Čepcová, C. P. Joschko, A. van Buiten, M. Goris, F. Hoogstra‐Berends, N. J. Kloosterhuis, N. Huijkman, B. van de Sluis, G. F. Diercks, J. H. Buikema, R. H. Henning, L. E. Deelman |
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| Přispěvatelé: | Center for Liver, Digestive and Metabolic Diseases (CLDM), Translational Immunology Groningen (TRIGR), Groningen Kidney Center (GKC), Groningen Institute for Organ Transplantation (GIOT), Restoring Organ Function by Means of Regenerative Medicine (REGENERATE) |
| Jazyk: | angličtina |
| Rok vydání: | 2022 |
| Předmět: |
inorganic chemicals
Aging congenital hereditary and neonatal diseases and abnormalities hydrogen sulfide Cystathionine beta-Synthase inducible mouse model HYDROGEN-SULFIDE PHENOTYPE Biochemistry DEFICIENT MICE DISEASE Mice CYSTATHIONINE-BETA-SYNTHASE Pregnancy RISK-FACTOR ANIMAL-MODEL Genetics Animals STEATOSIS FIBROSIS hyperhomocysteinemia Molecular Biology Mice Knockout organic chemicals nutritional and metabolic diseases cystathionine beta synthase deficiency Alopecia MOUSE MODEL vascular dysfunction Disease Models Animal Female Homocystinuria Biotechnology |
| Zdroj: | The FASEB Journal, 36(4):22260. FEDERATION AMER SOC EXP BIOL |
| ISSN: | 1530-6860 0892-6638 |
| Popis: | Deficiencies in Cystathionine-β-synthase (CBS) lead to hyperhomocysteinemia (HHCy), which is considered a risk factor for cardiovascular, bone and neurological disease. Moreover, CBS is important for the production of cysteine, hydrogen sulfide (H2 S) and glutathione. Studying the biological role of CBS in adult mice has been severely hampered by embryological disturbances and perinatal mortality. To overcome these issues and assess the effects of whole-body CBS deficiency in adult mice, we engineered and characterized a Cre-inducible Cbs knockout model during ageing. No perinatal mortality occurred before Cbs-/- induction at 10 weeks of age. Mice were followed until 90 weeks of age and ablation of Cbs was confirmed in liver and kidney but not in brain. Severe HHCy was observed in Cbs-/- (289 ± 58 µM) but not in Cbs+/- or control mice ( |
| Databáze: | OpenAIRE |
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