Till startsida
Webbkarta
Till innehåll Läs mer om hur kakor används på gu.se

Osteocyte Protein Expression Is Altered in Low-Turnover Osteoporosis Caused by Mutations in WNT1 and PLS3

Artikel i vetenskaplig tidskrift
Författare K. Wesseling-Perry
R. E. Makitie
V. V. Valimaki
Tero Laine
Christine M. Laine
M. J. Valimaki
R. C. Pereira
O. Makitie
Publicerad i Journal of Clinical Endocrinology & Metabolism
Volym 102
Nummer/häfte 7
Sidor 2340-2348
ISSN 0021-972X
Publiceringsår 2017
Publicerad vid Institutionen för kliniska vetenskaper, sektionen för anestesi, biomaterial och ortopedi, Avdelningen för ortopedi
Institutionen för medicin
Sidor 2340-2348
Språk English
Länkar dx.doi.org/10.1210/jc.2017-00099
Ämnesord early-onset osteoporosis, bone mass, parathyroid-hormone, fgf23, mice, osteoblastogenesis, histomorphometry, sclerostin, children, ablation
Ämneskategorier Endokrinologi och diabetes, Ortopedi, Invärtesmedicin

Sammanfattning

Context: Osteocytes express proteins that regulate bone remodeling and mineralization. Objective: To evaluate the relationship between osteocyte-specific protein expression and bone histology in patients with monogenic osteoporosis due to wingless integration site 1 (WNT1) or plastin 3 (PLS3) mutations. Design and Setting: Cross-sectional cohort study at a university hospital. Participants: Six patients (four males; ages: 14 to 72 years) with a heterozygous WNT1 mutation and five patients (four males; ages: 9 to 70 years) with a heterozygous/hemizygous PLS3 mutation. Methods and Main Outcome Measures: Immunohistochemistry was performed for fibroblast growth factor 23 (FGF23), dentin matrix protein 1 (DMP1), sclerostin, and phosphorylated (phospho-)beta-catenin in iliac crest samples and compared with bone histomorphometry. Results: FGF23 expression in WNT1 patients was 243% that observed in PLS3 patients (P < 0.01). DMP1, sclerostin, and phospho-beta-catenin expression did not differ between groups. Serum phosphate correlated inversely with FGF23 expression (r = -0.79, P = 0.01) and serum ionized calcium correlated inversely with sclerostin expression (r = -0.60, P = 0.05). Phospho-beta-catenin expression correlated inversely with DMP1 expression (r = -0.88, P < 0.001), osteoid volume/bone volume (r = -0.68, P = 0.02), and bone formation rate (r = -0.78, P < 0.01). FGF23 expression did not correlate with DMP1 expression, sclerostin expression, or bone histomorphometry. Marrow adiposity was higher in WNT1 than in PLS3 patients (P = 0.04). Conclusions: Mutations that disrupt WNT signaling and osteocytic mechanosensing affect osteocyte protein expression. Abnormal osteocyte function may play a role in the pathogenesis of monogenetic forms of osteoporosis.

Sidansvarig: Webbredaktion|Sidan uppdaterades: 2012-09-11
Dela:

På Göteborgs universitet använder vi kakor (cookies) för att webbplatsen ska fungera på ett bra sätt för dig. Genom att surfa vidare godkänner du att vi använder kakor.  Vad är kakor?