Faculty of Dentistry, Department of Physiology, Center of Excellence in Skeletal Disorders and Enzyme Reaction Mechanism, Chulalongkorn University, Bangkok, Thailand.
Faculty of Science, Thalassemia Research Center, Institute of Molecular Biosciences, Department of Biochemistry, Mahidol University, Bangkok, Thailand.
PLoS One. 2022 May 27;17(5):e0268732. doi: 10.1371/journal.pone.0268732. eCollection 2022.
Although patients with either β-thalassemia or chronic kidney disease (CKD) clinically correlate with severe osteoporosis, the mechanism by which CKD exposed to high phosphate affects bone turnover has not been characterized in β-thalassemia. We aimed to determine the effects of renal insufficiency on high phosphate intake induced changes in bone metabolism after 5/6th nephrectomy in hemizygous β-globin knockout (BKO) mice. Male BKO mice manifested severe anemia and osteopenia. Nephrectomy induced renal fibrosis and reduced renal function as assessed by increased serum urea nitrogen levels. Moreover, nephrectomy increased bone turnover leading to bone loss in wild type (WT) but not BKO mice. In nephrectomized BKO, PBS in drinking water induced hyperphosphatemia, and hypercalcemia along with osteopenia in both cancellous and cortical bone. Histomorphometric analysis confirmed reduced cancellous bone volume due to decreased bone formation rate, osteoblast number and osteoclast number. The mRNA levels for Alpl, Sp7, Kl, Tnfsf11, and Tnfsf11/Tnfrsf11b were decreased in nephrectomized BKO mice drinking PBS. Interestingly, Fgf23, a bone-derived hormone produced by osteocytes and osteoblasts in response to hyperphosphatemia, were remarkably increased in nephrectomized BKO mice following PBS intake. Serum FGF23 and erythropoietin levels were markedly elevated in BKO mice. Nephrectomy decreased serum erythropoietin but not FGF23 levels. Hyperphosphatemia in BKO mice increased serum erythropoietin, FGF23, and PTH levels, nominating these factors as candidate mediators of bone loss in thalassemic mice with CKD during phosphate retention.
虽然β-地中海贫血或慢性肾脏病 (CKD) 患者在临床上与严重骨质疏松症相关,但 CKD 暴露于高磷如何影响骨转换在β-地中海贫血中尚未得到描述。我们旨在确定肾功能不全对 5/6 肾切除术后高磷摄入诱导半β-球蛋白敲除 (BKO) 小鼠骨代谢变化的影响。雄性 BKO 小鼠表现出严重的贫血和骨质疏松症。肾切除诱导肾纤维化和肾功能降低,表现为血清尿素氮水平升高。此外,肾切除增加了骨转换,导致野生型 (WT) 但不是 BKO 小鼠的骨丢失。在肾切除的 BKO 中,PBS 在饮用水中诱导高磷血症、高钙血症以及皮质和松质骨的骨质疏松症。组织形态计量学分析证实,由于骨形成率、成骨细胞数量和破骨细胞数量减少,松质骨体积减少。饮用 PBS 的肾切除 BKO 小鼠的 Alpl、Sp7、Kl、Tnfsf11 和 Tnfsf11/Tnfrsf11b 的 mRNA 水平降低。有趣的是,成骨细胞和破骨细胞对高磷血症产生的骨源性激素 Fgf23 在 PBS 摄入后在肾切除的 BKO 小鼠中显著增加。BKO 小鼠的血清 FGF23 和促红细胞生成素水平显著升高。肾切除降低了血清促红细胞生成素但不降低 FGF23 水平。BKO 小鼠的高磷血症增加了血清促红细胞生成素、FGF23 和 PTH 水平,这表明这些因素是 CKD 期间磷酸盐潴留导致地中海贫血小鼠骨丢失的候选介质。