Bone matrix hypermineralization in prolyl-3 hydroxylase 1 deficient mice

被引：15

作者：

Fratzl-Zelman, Nadja ^{[1
,2
]}

Baechinger, Hans-Peter ^{[3
]}

Vranka, Janice A. ^{[4
]}

Roschger, Paul ^{[1
,2
]}

Klaushofer, Klaus ^{[1
,2
]}

Rauch, Frank ^{[5
,6
]}

机构：

[1] Hanusch Hosp WGKK, Ludwig Boltzmann Inst Osteol, Vienna, Austria

[2] Hanusch Hosp, Dept Med 1, AUVA Trauma Ctr Meidling, Vienna, Austria

[3] Shriners Hosp Children, Res Dept, Portland, OR 97201 USA

[4] Oregon Hlth & Sci Univ, Dept Ophtalmol, Portland, OR 97201 USA

[5] Shriners Hosp Children, 1529 Cedar Ave, Montreal, PQ H3G 1A6, Canada

[6] McGill Univ, Montreal, PQ, Canada

来源：

BONE | 2016年 / 85卷

关键词：

Bone histomorphometry; Bone mineralization density distribution; Murine bone; Prolyl-3; hydroxylase-1; deficiency; Quantitative backscattered electron imaging; RECESSIVE OSTEOGENESIS IMPERFECTA; MINERALIZATION DENSITY DISTRIBUTION; CORTICAL BONE; BRITTLE BONE; MOUSE MODEL; MUTATION; CHILDREN; DISEASE; CRTAP; HISTOMORPHOMETRY;

D O I：

10.1016/j.bone.2016.01.018

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Lack of prolyl 3-hydroxylase 1 (P3H1) due to mutations in P3H1 results in severe forms of recessive osteogenesis imperfecta. In the present study, we investigated the bone tissue characteristics of P3H1 null mice. Histomorphometric analyses of cancellous bone in the proximal tibia and lumbar vertebra in 1-month and 3-month old mice demonstrated that P3H1 deficient mice had low trabecular bone volume and low mineral apposition rate, but normal osteoid maturation time and normal osteoblast and osteoclast surfaces. Quantitative backscattered electron imaging revealed that the bone mineralization density distribution was shifted towards higher values, indicating hypermineralization of bone matrix. It thus appears that P3H1 deficiency leads to decreased deposition of extracellular matrix by osteoblasts and increased incorporation of mineral into the matrix. (C) 2016 Elsevier Inc. All rights reserved.

引用

页码：15 / 22

页数：8

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