scholarly journals CRTAP deficiency leads to abnormally high bone matrix mineralization in a murine model and in children with osteogenesis imperfecta type VII

Bone ◽  
2010 ◽  
Vol 46 (3) ◽  
pp. 820-826 ◽  
Author(s):  
N. Fratzl-Zelman ◽  
R. Morello ◽  
B. Lee ◽  
F. Rauch ◽  
F.H. Glorieux ◽  
...  
Keyword(s):  
Osteogenesis Imperfecta ◽  
Murine Model ◽  
Bone Matrix ◽  
Osteogenesis Imperfecta Type ◽  
Matrix Mineralization ◽  
High Bone ◽  
Bone Matrix Mineralization

CRTAP deficiency leads to high bone matrix mineralization in children (Osteogenesis imperfecta type VII) and mice

Bone ◽  
2009 ◽  
Vol 44 ◽  
pp. S232-S233
Author(s):  
N. Fratzl-Zelman ◽  
R. Morello ◽  
B. Lee ◽  
F. Rauch ◽  
F.H. Glorieux ◽  
...  
Keyword(s):  
Osteogenesis Imperfecta ◽  
Bone Matrix ◽  
Osteogenesis Imperfecta Type ◽  
Matrix Mineralization ◽  
High Bone ◽  
Bone Matrix Mineralization

Bone matrix mineralization in osteogenesis imperfecta type VI

Bone ◽  
2012 ◽  
Vol 51 (6) ◽  
pp. S14
Author(s):  
N. Fratzl-Zelman ◽  
P. Roschger ◽  
I. Schmidt ◽  
F. Glorieux ◽  
K. Klaushofer ◽  
...  
Keyword(s):  
Osteogenesis Imperfecta ◽  
Bone Matrix ◽  
Osteogenesis Imperfecta Type ◽  
Matrix Mineralization ◽  
Bone Matrix Mineralization

Bone matrix mineralization after sclerostin antibody treatment in a mouse model of osteogenesis imperfecta

2014 ◽  
Author(s):  
Andreas Roschger ◽  
Paul Roschger ◽  
Michaela Kneissel ◽  
Frank Rauch
Keyword(s):  
Mouse Model ◽  
Osteogenesis Imperfecta ◽  
Bone Matrix ◽  
Antibody Treatment ◽  
Matrix Mineralization ◽  
Sclerostin Antibody ◽  
Bone Matrix Mineralization

Novel familial mutation of LRP5 causing high bone mass: Genetic analysis, clinical presentation, and characterization of bone matrix mineralization

Bone ◽  
2018 ◽  
Vol 107 ◽  
pp. 154-160 ◽  
Author(s):  
K.M. Roetzer ◽  
G. Uyanik ◽  
A. Brehm ◽  
J. Zwerina ◽  
S. Zandieh ◽  
...  
Keyword(s):  
Genetic Analysis ◽  
Bone Mass ◽  
Clinical Presentation ◽  
Bone Matrix ◽  
High Bone Mass ◽  
Matrix Mineralization ◽  
High Bone ◽  
Bone Matrix Mineralization

Rescue diet restores bone matrix mineralization in mice with a non-functioning vitamin D receptor

2019 ◽  
Author(s):  
Barbara Misof ◽  
Stephane Blouin ◽  
Markus Hartmann ◽  
Jochen Hofstaetter ◽  
Klaus Klaushofer ◽  
...  
Keyword(s):  
Vitamin D ◽  
Vitamin D Receptor ◽  
Bone Matrix ◽  
Matrix Mineralization ◽  
Bone Matrix Mineralization

Normal bone matrix mineralization in patients with chronic obstructive pulmonary disease

2014 ◽  
Author(s):  
Barbara Misof ◽  
Paul Roschger ◽  
Vanda Jorgetti ◽  
Klaus Klaushofer ◽  
David Dempster ◽  
...  
Keyword(s):  
Chronic Obstructive Pulmonary Disease ◽  
Pulmonary Disease ◽  
Bone Matrix ◽  
Chronic Obstructive ◽  
Matrix Mineralization ◽  
Obstructive Pulmonary Disease ◽  
Normal Bone ◽  
Bone Matrix Mineralization

A novel transgenic murine model with persistently brittle bones simulating osteogenesis imperfecta type I

Bone ◽  
2019 ◽  
Vol 127 ◽  
pp. 646-655 ◽  
Author(s):  
Yi Liu ◽  
Jianhai Wang ◽  
Shuo Liu ◽  
Mingjie Kuang ◽  
Yaqing Jing ◽  
...  
Keyword(s):  
Osteogenesis Imperfecta ◽  
Murine Model ◽  
Type I ◽  
Osteogenesis Imperfecta Type ◽  
Transgenic Murine Model ◽  
Brittle Bones ◽  
Osteogenesis Imperfecta Type I

Changes in Bone Matrix Mineralization After Growth Hormone Treatment in Children and Adolescents With Chronic Kidney Failure Treated by Dialysis: A Paired Biopsy Study

2013 ◽  
Vol 61 (5) ◽  
pp. 767-777 ◽  
Author(s):  
Kamilla Nawrot-Wawrzyniak ◽  
Barbara M. Misof ◽  
Paul Roschger ◽  
Małgorzata Pańczyk-Tomaszewska ◽  
Helena Ziółkowska ◽  
...  
Keyword(s):  
Growth Hormone ◽  
Children And Adolescents ◽  
Kidney Failure ◽  
Growth Hormone Treatment ◽  
Bone Matrix ◽  
Chronic Kidney Failure ◽  
Hormone Treatment ◽  
Matrix Mineralization ◽  
Bone Matrix Mineralization

scholarly journals Osteoblastic Response to the Defective Matrix in the Osteogenesis Imperfecta Murine (oim) Mouse

Endocrinology ◽  
2002 ◽  
Vol 143 (5) ◽  
pp. 1594-1601 ◽  
Author(s):  
I. Kalajzic ◽  
J. Terzic ◽  
Z. Rumboldt ◽  
K. Mack ◽  
A. Naprta ◽  
...  
Keyword(s):  
Bone Resorption ◽  
Osteogenesis Imperfecta ◽  
Bone Matrix ◽  
High Rate ◽  
Transferase Activity ◽  
Osteoblast Activity ◽  
Chloramphenicol Acetyl Transferase Activity ◽  
High Bone ◽  
Normal Quantity ◽  
And Control

Abstract This work examines the cellular pathophysiology associated with the weakened bone matrix found in a murine model of osteogenesis imperfecta murine (oim). Histomorphometric analysis of oim/oim bone showed significantly diminished bone mass, and the osteoblast and osteoclast histomorphometric parameters were increased in the oim/oim mice, compared with wild-type (+/+) mice. To assess osteoblast activity, a rat Col1a1 promoter linked to the chloramphenicol acetyltransferase reporter transgene was bred into the oim model. At 8 d and 1 month of age, no difference in transgene activity between oim and control mice was observed. However, at 3 months of age, chloramphenicol acetyl transferase activity was elevated in oim/oim;Tg/Tg, compared with +/+;Tg/Tg and oim/+;Tg/Tg. High levels of urinary pyridinoline crosslinks in the oim/oim;Tg/Tg mice were present at all ages, reflecting continuing high bone resorption. Our data portray a state of ineffective osteogenesis in which the mutant mouse never accumulates a normal quantity of bone matrix. However, it is only after the completion of the rapid growth phase that the high activity of the oim/oim osteoblast can compensate for the high rate of bone resorption. This relationship between bone formation and resorption may explain why the severity of osteogenesis imperfecta decreases after puberty is completed. The ability to quantify high bone turnover and advantages of using a transgene that reflects osteoblast lineage activity make this a useful model for studying interventions designed to improve the bone strength in osteogenesis imperfecta.

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