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Runx2/Cbfa1杂合小鼠的牙齿萌出和牙骨质形成

Tooth eruption and cementum formation in the Runx2/Cbfa1 heterozygous mouse.

作者信息

Zou S J, D'Souza R N, Ahlberg T, Bronckers A L J J

机构信息

Department of Oral Cell Biology, ACTA, van der Boechorststr 7, 1081 BT Amsterdam, The Netherlands.

出版信息

Arch Oral Biol. 2003 Sep;48(9):673-7. doi: 10.1016/s0003-9969(03)00135-3.

Abstract

Cleidocranial dysplasia (CCD) is an autosomal dominant human disorder that affects development of bones and teeth. The dental disorders in CCD patients include formation of supernumerary teeth, delayed tooth eruption, and lack of formation of cellular cementum in permanent teeth. This disorder involves a mutation in the osteoblast-specific transcription factor Runx2/Cbfa1, leading to haploinsufficiency of the Runx2/Cbfa1 protein. Here, we examined if Runx2/Cbfa1 heterozygous mice (with one functional allele for Runx2/Cbfa1) exhibit similar changes in tooth eruption, and dental cementum formation as in CCD patients. Heads of Runx2/Cbfa1 heterogeneous and wildtype mice aged days 16-35 postnatally were serially sectioned and stained with hematoxylin-eosin or for tartrate resistant acid phosphatase (TRAP) to identify osteoclasts. The results showed that the eruption pattern of the first and second molars in maxilla and mandible in Runx2/Cbfa1 +/- mice was the same as in wildtype animals. No clear difference in distribution or in the (estimated) number of osteoclasts was found. Cellular cement at the apical portions of the molar roots was present in both groups. The data suggests that in the mouse one allele for Runx2/Cbfa1 is sufficient for an undisturbed tooth eruption and an apparently normal formation of the periodontium.

摘要

锁骨颅骨发育不全(CCD)是一种常染色体显性人类疾病,会影响骨骼和牙齿的发育。CCD患者的牙齿疾病包括多生牙形成、牙齿萌出延迟以及恒牙中细胞性牙骨质形成缺失。这种疾病涉及成骨细胞特异性转录因子Runx2/Cbfa1的突变,导致Runx2/Cbfa1蛋白单倍体不足。在此,我们研究了Runx2/Cbfa1杂合小鼠(具有一个功能性Runx2/Cbfa1等位基因)是否表现出与CCD患者类似的牙齿萌出和牙骨质形成变化。对出生后16 - 35天的Runx2/Cbfa1杂合和野生型小鼠的头部进行连续切片,并用苏木精 - 伊红或抗酒石酸酸性磷酸酶(TRAP)染色以识别破骨细胞。结果表明,Runx2/Cbfa1 +/-小鼠上颌和下颌第一和第二磨牙的萌出模式与野生型动物相同。在破骨细胞的分布或(估计)数量上未发现明显差异。两组磨牙牙根顶端均存在细胞性牙骨质。数据表明,在小鼠中,一个Runx2/Cbfa1等位基因足以实现牙齿的正常萌出和牙周组织的明显正常形成。

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