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Title: Bone microstructural defects and osteopenia in hemizygous β<sup>IVSII-654</sup>knockin thalassemic mice: Sex-dependent changes in bone density and osteoclast function
Authors: Kanogwun Thongchote
Saovaros Svasti
Jarinthorn Teerapornpuntakit
Panan Suntornsaratoon
Nateetip Krishnamra
Narattaphol Charoenphandhu
Mahidol University
Keywords: Biochemistry, Genetics and Molecular Biology;Medicine
Issue Date: 1-Jan-2015
Citation: American Journal of Physiology - Endocrinology and Metabolism. Vol.309, No.11 (2015), E936-E948
Abstract: © 2015 the American Physiological Society. β-Thalassemia, a hereditary anemic disorder, is often associated with skeletal complications that can be found in both males and females. The present study aimed to investigate the age- and sex-dependent changes in bone mineral density (BMD) and trabecular microstructure in βIVSII-654knockin thalassemic mice. Dual-energy X-ray absorptiometry and computer-assisted bone histomorphometry were employed to investigate temporal changes in BMD and histomorphometric parameters in male and female mice of a βIVSII-654knockin mouse model of human β-thalassemia, in which impaired splicing of β-globin transcript was caused by hemizygous C→T mutation at nucleotide 654 of intron 2. Young, growing βIVSII-654mice (1 mo old) manifested shorter bone length and lower BMD than their wild-type littermates, indicating possible growth retardation and osteopenia, the latter of which persisted until 8 mo of age (adult mice). Interestingly, two-way analysis of variance suggested an interaction between sex and βIVSII-654genotype, i.e., more severe osteopenia in adult female mice. Bone histomorphometry further suggested that low trabecular bone volume in male βIVSII-654mice, particularly during a growing period (1–2 mo), was primarily due to suppression of bone formation, whereas both a low bone formation rate and a marked increase in osteoclast surface were observed in female βIVSII-654mice. In conclusion, osteopenia and trabecular microstructural defects were present in both male and female βIVSII-654knockin thalassemic mice, but the severity, disease progression, and cellular mechanism differed between the sexes.
ISSN: 15221555
Appears in Collections:Scopus 2011-2015

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