Defective bone formation by hyp mouse bone cells transplanted into normal mice: Evidence in favor of an intrinsic osteoblast defect

Defective bone formation by hyp mouse bone cells transplanted into normal mice: Evidence in favor of an intrinsic osteoblast defect
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hyp小鼠骨细胞移植到正常小鼠体内后形成有缺陷的骨:支持内在成骨细胞缺陷的证据

DOI:
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发表时间:
1992
影响因子:
6.2
通讯作者:
L. Labelle
L. Labelle
中科院分区:
医学1区
文献类型:
--
作者:
B. Ecarot;F. Glorieux;M. Desbarats;R. Travers;L. Labelle

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低磷酸盐血症(Hyp)小鼠是人类低磷酸盐血症维生素D抵抗性佝偻病的动物模型。我们曾报道,从纯合突变雌性Hyp小鼠中分离的骨细胞移植到正常小鼠中时会产生异常骨。为了测试突变细胞的环境获得性缺陷是否有助于在移植物中观察到的骨形成受损,将来自正常和Hyp同窝仔的骨膜和成骨细胞肌内移植到正常动物中。为了在移植前更具体地测试低磷酸盐血症诱导的细胞改变,将从磷酸盐耗尽的正常小鼠中分离的骨细胞移植到正常动物中。通过测量其类骨质厚度和体积来表征在2周移植物中形成的骨结节。通过类骨质厚度和体积的增加,与正常同窝移植相比,Hyp移植证明骨形成受损。与来自突变小鼠的细胞相反,从具有可比低磷酸盐血症的正常小鼠分离的细胞产生正常的骨。这些结果表明,Hyp成骨细胞在正常环境中不能产生正常骨不是先前暴露于改变的环境的结果,而是可能是内在的细胞异常。这些观察结果进一步支持了成骨细胞是Hyp突变的重要靶点的概念。
The hypophosphatemic (Hyp) mouse is an animal model for human hypophosphatemic vitamin D‐resistant rickets. We have reported that bone cells isolated from Hyp mice born to homozygous mutant females produce abnormal bone when transplanted into normal mice. To test whether an environmentally acquired defect of the mutant cells contributed to the impaired bone formation observed in transplants, periostea and osteoblasts from normal and Hyp littermates were transplanted intramuscularly into normal animals. To test more specifically for an hypophosphatemia‐induced cell alteration before transplantation, bone cells isolated from phosphate‐depleted normal mice were transplanted into normal animals. The bone nodules formed in 2 week transplants were characterized by measuring their osteoid thickness and volume. Impaired bone formation was evidenced in Hyp transplants compared to normal littermate transplants by increased osteoid thickness and volume. In contrast to cells from mutant mice, cells isolated from normal mice with comparable hypophosphatemia produced normal bone. These results indicate that the inability of Hyp osteoblasts to produce normal bone when placed in a normal environment is not the consequence of prior exposure to an altered environment but likely of an intrinsic cellular abnormality. These observations add further support to the concept that the osteoblast is an important target for the Hyp mutation.
甲状旁腺激素对 X 连锁低磷血症性佝偻病患者血清 1,25-二羟基维生素 D 水平的影响:25-羟基维生素 D-1-羟化酶活性异常的证据。
DOI: 10.1210/jcem-54-3-638
发表时间: 1982
期刊: The Journal of clinical endocrinology and metabolism
影响因子: --
作者:
Lyles,KW;Drezner,MK
通讯作者: Drezner,MK
X 连锁低磷血症小鼠中维生素 D 代谢异常。
DOI: 10.1210/endo-107-5-1577
发表时间: 1980
期刊: Endocrinology
影响因子: 4.8
作者:
MeyerJr,RA;Gray,RW;Meyer,MH
通讯作者: Meyer,MH
异常腺苷 3.5-单磷酸刺激 hyp 小鼠肾脏 1,25-二羟基维生素 D 的产生:证据表明 25-羟基维生素 D-1 α-羟化酶功能障碍是由异常的细胞内功能引起的。
DOI: 10.1210/endo-124-3-1184
发表时间: 1989
期刊: Endocrinology
影响因子: 4.8
作者:
Nesbitt,T;Davidai,GA;Drezner,MK
通讯作者: Drezner,MK
X 连锁低磷血症小鼠肾脏 25-羟基维生素 D-1 α-羟化酶活性的异常调节。
DOI: 10.1172/jci110783
发表时间: 1983
期刊: The Journal of clinical investigation
影响因子: --
作者:
Lobaugh,B;Drezner,MK
通讯作者: Drezner,MK