• Journal of The Korean Society of Inherited Metabolic disease
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• v.16 no.3
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• pp.141-147
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• 2016

Hypophosphatasia is caused by the mutations in ALPL, which encodes tissue-nonspecific alkaline phosphatase (TNSALP). It can be inherited either in an autosomal dominant or recessive manner. Clinically, hypophophosphatasia is characterized by skeletal findings similar to those in rickets or osteomalacia, but serum alkaline phosphatase levels are decreased in the affected patients. Hypophosphatasia can be classified into six clinical forms according to age at diagnosis and severity of symptoms: perinatal lethal, infantile, childhood, adult, odontohypophosphatasia, and perinatal benign. As being a very rare disease, only one case has been reported in Korean population. Here we describe a case with perinatal benign hypophosphatasia with recessive ALPL mutations. Bowing of lower legs was detected in prenatal period and low serum alkaline phosphatase level was noted after birth. During the follow-up evaluation for 4.5 years, bone mineralization and legs bowing were improved but the growth retardation was persistent. As the recombinant bone-targeted human TNSALP became available, the clinical improvement of the affected patients is expected including the case described here with this treatment. More efforts are needed to identify the cases affected by hypophosphatasia.

• The Korea Journal of Herbology
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• v.36 no.5
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• pp.81-91
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• 2021

Objectives : Osteoporosis is a systemic skeletal disease that decreases bone density and increases the risk of fractures. Bisphosphonates and SERMs are mainly used to treat osteoporosis, but, long-term use increases the risk of side effects such as jaw bone necrosis and breast cancer. Therefore, it is necessary to develop a therapeutic agent for a natural product with few side effects. Water extract of Lonicerae Japonicae Flos (wLF) was mainly found to have anti-cancer and anti-inflammatory effects. However, the effect of wLF on osteoporosis has not been elucidated. Therefore, this experiment investigated the effect of wLF on osteoclasts, osteoblasts and osteoporosis models. Methods : In order to study the effect of wLF on osteoporosis, the OVX-induced rat model was used for in vivo study. After 8 weeks, we measured body weight, uterine weight, liver weight, femur weight, bone density, trabecular area and tibia ash weight. To determine the effect of wLF on osteoclast differentiation, we measured the number of TRAP-positive cells and TRAP activity. To examine the effect of wLF on the expression of osteoblast-related genes, we measured the mRNA expression of alkaline phosphatase (ALP, Alpl) and osteocalcin (OCN, Bglap2). Results : In vivo experiment, wLF inhibited the reduction of femur weight, trabecular area, bone density and tibia ash weight. In vitro experiment, wLF had no significant effect on osteoclast differentiation. However, wLF increased the mRNA expression of Alpl and Bglap2 in MC3T3-E1 cell. Conclusions : This result suggested that wLF may be used for the treatment and prevention of postmenopausal osteoporosis.

• Journal of Korean Dental Science
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• v.9 no.1
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• pp.9-18
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• 2016

Purpose: Wnt signaling plays an essential role in the dental epithelium and mesenchyme during tooth morphogenesis. Deletion of the Wntless (Wls) gene in odontoblasts appears to reduce canonical Wnt activity, leading to inhibition of odontoblast maturation. However, it remains unclear if autonomous Wnt ligands are necessary for differentiation of dental pulp cells into odontoblast-like cells to induce reparative dentinogenesis, one of well-known feature of pulp repair to form tertiary dentin. Materials and Methods: To analyze the autonomous role of Wls for differentiation of dental pulp cells into odontoblast-like cells, we used primary dental pulp cells from unerupted molars of Wls-floxed allele mouse after infection with adenovirus for Cre recombinase expression to knockout the floxed Wls gene or control GFP expression. The differentiation of dental pulp cells into odontoblast-like cells was analyzed by quantitative real-time polymerase chain reaction. Result: Proliferation rate was significantly decreased in dental pulp cells with Cre expression for Wls knockout. The expression levels of Osterix (Osx), runt-related transcription factor 2 (Runx2), and nuclear factor I-C (Nfic) were all significantly decreased by 0.3-fold, 0.2-fold, and 0.3-fold respectively in dental pulp cells with Wls knockout. In addition, the expression levels of Bsp, Col1a1, Opn, and Alpl were significantly decreased by 0.7-fold, 0.3-fold, 0.8-fold, and 0.6-fold respectively in dental pulp cells with Wls knockout. Conclusion: Wnt ligands produced autonomously are necessary for proper proliferation and odontoblastic differentiation of mouse dental pulp cells toward further tertiary dentinogenesis.

• Journal of Life Science
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• v.28 no.12
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• pp.1448-1454
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• 2018

Insects have been investigated as a novel source of food and biomaterial in several recent studies. However, their osteoblastogenic cell activity has not been sufficiently researched and so, to investigate the potential of this natural material for promoting osteoblastogenesis, we studied the activity of Locusta migratoria ethanol extract (LME) on MG-63 pre-osteoblast cells. The cytotoxicity and proliferation effects of LME on MG-63 cells were measured by MTS assay, and there was no cytotoxicity up to $1,000{\mu}g/ml$. With LME treatment of 500 and $1,000{\mu}g/ml$ for 48 hr, cell proliferation increased to 105% and 116% versus control, respectively. The osteoblastogenic activity of the LME was measured through alkaline phosphatase (ALP) staining at three and five days. As a result, both 500 and $1,000{\mu}g/ml$ LME concentrations were seen to increase ALP activity by more than three times compared with control at three and five days. In addition, the expression level of the osteogenic markers ALP and RUNX2 was markedly increased after LME treatment. These results demonstrate that Locusta migratoria ethanol extract promotes osteoblastogenesis as evidenced by the increased osteogenic markers and suggest that LME may be a potential agent for bone formation and osteoporosis prevention.

• Journal of Life Science
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• v.29 no.9
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• pp.1027-1033
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• 2019

Recently, Korea has seen a rapid increase in the elderly population. As a result, osteoporosis, a geriatric disease, has become a social problem. To investigate the novel and natural materials for promoting osteoblastogenesis, we investigated the osteoblastogenic activity of Tenebrio molitor larvae oil (TMO) on the MG-63 preosteoblast cells. The cytotoxicity and proliferation effects of TMO on MG-63 cells were measured by MTS assay. There was no cytotoxicity up to $80{\mu}g/ml$. At 40 and $80{\mu}g/ml$ of TMO (treated for 48 hr), cell proliferation was elevated about 120% compared to the control. The osteoblastogenic activity of TMO was measured with alkaline phosphatase (ALP) activity at 5 days. Doses of 5 to $80{\mu}g/ml$ of TMO increased ALP activities significantly compared with the control. In addition, expression of ALP and Runx2 (osteoblastogenic markers) were markedly increased after treatment of TMO for 5 days. These results provide evidence that TMO promotes osteoblastogenesis by increasing the gene and protein expression of ALP and Runx2, and they suggest that TMO may be a potential agent for bone formation and preventing osteoporosis.