Abonnement à la biblothèque: Guest
Portail numérique Bibliothèque numérique eBooks Revues Références et comptes rendus Collections
Critical Reviews™ in Eukaryotic Gene Expression
Facteur d'impact: 2.156 Facteur d'impact sur 5 ans: 2.255 SJR: 0.649 SNIP: 0.599 CiteScore™: 3

ISSN Imprimer: 1045-4403
ISSN En ligne: 2162-6502

Critical Reviews™ in Eukaryotic Gene Expression

DOI: 10.1615/CritRevEukaryotGeneExpr.v13.i24.90
10 pages

Serum Levels of TGF-b and Fibronectin in Autosomal Dominant Osteopetrosis in Relation to Underlying Mutations and Well-Described Murine Counterparts

Jens Bollerslev
Department of Endocrinology, National University Hospital, N-0027 Oslo, Norway; and School of Medicine and Pharmacology, University of Western Australia, Nedlands, 6009 WA, Australia
Thor Ueland
Department of Endocrinology and Research Institute for Internal Medicine, National University Hospital, N-0027 Oslo, Norway
Paul R. Odgren
Dept. of Cell Biology, University of Massachusetts Medical School, Worcester, MA 01655


The study gives a further biochemical description of two different forms of autosomal dominant osteopetrosis (ADO) in relation to murine counterparts, with special attention to osteoblast function and the recent discovery of LRP5 gene mutations in ADO I. Patients and controls were investigated for markers of bone formation and resorption at baseline and following stimulation with thyroid hormone. Moreover, four different well-described murine models of osteopetrosis were investigated.
Concerning the human forms, serum TSH levels decreased in all subjects, indicating effects on the target tissue. Osteocalcin and cross-linked collagen (NTx) were without significant differences among the groups. Significant increases in both markers were seen following stimulation. Baseline active TGF-b1 levels were increased in both types of ADO (60% in ADO I [P = 0.006]; 46% in ADO II [P = 0.001], respectively), whereas fibronectin levels were decreased in both (ADO I 58% and ADO II 63% of normal, respectively [P = 0.012 and P = 0.001]). Following treatment, levels increased temporarily in all groups. In the murine models, active TGF-b1 was significantly decreased in the tl- and ia-rat, whereas fibronectin levels were decreased in the mi-mouse, however, increased in the ia-rat.
In conclusion, both types of ADO showed the same qualitative biochemical differences compared to controls, except that OPG levels were higher in ADO I. The decreased fibronectin levels in both types and in murine models reflect decreased bone resorption; however, this may also indicate hitherto unrecognized alterations in bone formation. Biochemical differences among known syndromes related to mutations in the LRP5 gene indicate different underlying pathogenetic mechanisms.

Articles with similar content:

Reactive Oxygen Species, Antioxidant Mechanisms, and Serum Cytokine Levels in Cancer Patients: Impact of an Antioxidant Treatment
Journal of Environmental Pathology, Toxicology and Oncology, Vol.22, 2003, issue 1
Clelia Madeddu, Loredana Mura, Elena Massa, Viviana Murgia, Giulia Gramignano, Giovanni Mantovani, Maria Rita Lusso, Paolo Camboni, Luca Ferreli, Antonio Maccio
Carcinogenicity and Chronic Toxicity of Para-Chloronitrobenzene in Rats and Mice by Two-Year Feeding
Journal of Environmental Pathology, Toxicology and Oncology, Vol.25, 2006, issue 3
Kazunori Yamazaki, Taijiro Matsushima, Seigo Yamamoto, Shigetoshi Aiso, Heihachiro Arito, Kasuke Nagano, Hideki Senoh, Michiharu Matsumoto
A Phase I/II Study of Ling Zhi Mushroom Ganoderma lucidum (W.Curt.:Fr.) Lloyd (Aphyllophoromycetideae) Extract in Patients with Coronary Heart Disease
International Journal of Medicinal Mushrooms, Vol.6, 2004, issue 4
Guoliang Chen, Jingxian Ye, Yihuai Gao, Shufeng Zhou, Xihu Dai
Insecticidal Activities of Ling Zhi or Reishi Medicinal Mushroom, Ganoderma lucidum (W. Curt.: Fr.) P. Karst. (Aphyllophoromycetideae) Extract against Tribolium castaneum and Drosophila melanogaster
International Journal of Medicinal Mushrooms, Vol.11, 2009, issue 3
Fatema Sargazi, Hossein Riahi, Masood Sheidai
Expression of p53 and Lactate Dehydrogenase in Murine Fibrosarcoma Following Exposure to g-Radiation
Journal of Environmental Pathology, Toxicology and Oncology, Vol.20, 2001, issue 1
Khalida Wani, Rema Rajagopalan, Nagaraj G. Huilgol, Cherupally Krishnan Krishnan Nair