Department of Medicine, McGill University and McGill University Health Centre, Montreal, Quebec, Canada.
Departments of Surgery and Human Genetics, McGill University, Montreal, Quebec, Canada.
JCI Insight. 2019 Jul 11;4(13). doi: 10.1172/jci.insight.126467.
We induced chronic kidney disease (CKD) with adenine in WT mice, mice with osteocyte-specific deletion of Cyp27b1, encoding the 25-hydroxyvitamin D 1(OH)ase [Oct-1(OH)ase-/-], and mice with global deletion of Cyp27b1 [global-1α(OH)ase-/-]; we then compared extraskeletal calcification. After adenine treatment, mice displayed increased blood urea nitrogen, decreased serum 1,25(OH)2D, and severe hyperparathyroidism. Skeletal expression of Cyp27b1 and of sclerostin and serum sclerostin all increased in WT mice but not in Oct-1α(OH)ase-/- mice or global-1α(OH)ase-/- mice. In contrast, skeletal expression of BMP2 and serum BMP2 rose in the Oct-1α(OH)ase-/- mice and in the global-1α(OH)ase-/- mice. Extraskeletal calcification occurred in muscle and blood vessels of mice with CKD and was highest in Oct-1α(OH)ase-/-mice. In vitro, recombinant sclerostin (100 ng/mL) significantly suppressed BMP2-induced osteoblastic transdifferentiation of vascular smooth muscle A7r5 cells and diminished BMP2-induced mineralization. Our study provides evidence that local osteocytic production of 1,25(OH)2D stimulates sclerostin and inhibits BMP2 production in murine CKD, thus mitigating osteoblastic transdifferentiation and mineralization of soft tissues. Increased osteocytic 1,25(OH)2D production, triggered by renal malfunction, may represent a "primary defensive response" to protect the organism from ectopic calcification by increasing sclerostin and suppressing BMP2 production.
我们用腺嘌呤诱导 WT 小鼠、骨细胞特异性 Cyp27b1 缺失(编码 25-羟维生素 D1(OH)酶[Oct-1(OH)ase-/-])的小鼠和 Cyp27b1 全局缺失(global-1α(OH)ase-/-)的小鼠发生慢性肾病(CKD),然后比较了其骨骼外钙化情况。腺嘌呤治疗后,小鼠出现血尿素氮升高、血清 1,25(OH)2D 降低和严重甲状旁腺功能亢进。WT 小鼠骨骼 Cyp27b1 及其硬骨素和血清硬骨素的表达均增加,但 Oct-1α(OH)ase-/- 小鼠或 global-1α(OH)ase-/- 小鼠则不然。相比之下,骨细胞 BMP2 的表达和血清 BMP2 水平在 Oct-1α(OH)ase-/- 小鼠和 global-1α(OH)ase-/- 小鼠中升高。CKD 小鼠的肌肉和血管出现骨骼外钙化,且在 Oct-1α(OH)ase-/- 小鼠中最为严重。体外,重组硬骨素(100ng/mL)显著抑制 BMP2 诱导的血管平滑肌 A7r5 细胞成骨细胞转分化,并减弱 BMP2 诱导的矿化。我们的研究提供了证据,即局部成骨细胞产生的 1,25(OH)2D 刺激硬骨素的产生并抑制 CKD 中小鼠 BMP2 的产生,从而减轻软组织的成骨细胞转分化和矿化。肾脏功能障碍引发的成骨细胞 1,25(OH)2D 产生增加,可能代表一种“初级防御反应”,通过增加硬骨素和抑制 BMP2 产生来保护机体免受异位钙化。