Mice lacking cystathionine beta synthase have lung fibrosis and air space enlargement

J Hamelet, N Maurin, R Fulchiron, JM Delabar… - … and molecular pathology, 2007 - Elsevier
Cystathionine beta synthase (CBS) is a crucial regulator of plasma concentrations of
homocysteine. Severe hyperhomocysteinemia due to CBS deficiency confers diverse
clinical manifestations, notably pulmonary thrombotic disease. However, the association
between hyperhomocysteinemia and chronic obstructive pulmonary disease is not well
understood. To investigate the role of hyperhomocysteinemia in lung injury and pulmonary
fibrosis, we analyzed the lung of CBS-deficient mice, a murine model of severe …
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