Homozygous whole body Cbs knockout in adult mice features minimal pathology during ageing despite severe homocysteinemia
- Nakladal, D
- Lambooy, S P H
- Mišúth, S
- Čepcová, D
- Joschko, C P
- van Buiten, A
- Goris, M
- Hoogstra-Berends, F
- Kloosterhuis, N J
- Huijkman, N
- van de Sluis, B
- Diercks, G F
- Buikema, J H
- Henning, R H
- Deelman, L E
DOIAbstract
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 ...
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