• Journal of The Korean Society of Inherited Metabolic disease
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• v.23 no.1
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• pp.1-11
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• 2023

Inborn errors of metabolism encompass a wide variety of disorders, frequently affecting bone. This review presents a comprehensive retrospect on the primary involvement of bone in inborn errors of metabolism. Primary involvement of bone in inborn errors of metabolism includes entities that primarily affect the bone marrow, mineral component or cartilage. These include lysosomal storage disorders, hypophosphatasia, and hereditary hypophosphatemic rickets. In this review, we discuss the primary involvement of bone in inborn errors of metabolism (hypophosphatasia, X-linked hypophosphatemic rickets, Gaucher disease, and mucopolysaccharidoses) along with the therapeutic agents used in clinical settings, diagnostic strategies, and general management. With the development of disease-specific targeted therapies and supportive care, more number of patients with these disorders live longer and survive into adulthood. Moreover, skeletal symptoms have become a more prominent feature of these disorders. This makes the awareness of these skeletal symptoms more important.

• Journal of the Korean Society of Food Science and Nutrition
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• v.26 no.3
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• pp.480-487
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• 1997

The present study was designed to examine how Ca intake contributes to the increase of peak bone mass with growing female rats. Weaned rats were fed experimental diets consisting in five levels of Ca; very low(0.1%), low(0.2%), moderate(0.5%), high(1.0%) and very high(1.5%) for 4, 8 and 12 weeks. Bone growth, metabolism and Ca metabolism were determined. As for the rats fed for 4 weeks, the bone weight, length and breaking force and bone metabolism were not significantly affected by dietary Ca levels, whereas the current intakes of Ca were observed to have significantly affected the rats fed for 8 or 12 weeks with regard to the bone weight, length and breaking force and bone metabolism. The bone ash and Ca contents of the rats were affected by dietary Ca levels for the total period of feeding. It is suggested that dietary Ca itself affected the mineralization process either during the growth or later, although the resulting bone mass is not a linear function of dietary Ca content.

• The Journal of Korean Physical Therapy
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• v.14 no.4
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• pp.412-422
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• 2002

The use of biochemical markers of bone turnover may be particular interest in the investigation of bone disorders with osteoporosis. Serum osteocalcin(OC), total alkaline phosphatase and procollagen C, reflecting bone formation, and urinary pyridinium cross-links excretion, reflecting bone reabsorption have been measured in hyperthyroidism, postmenopause women, after testosterone supplementation, androgen, testosterone and estrogen deficiency, bone mineral density degree, age duration. Bone marks which is reflect to metabolic bone disorders are biochemical indices method to measure enzyme activity about bone formation, bone absorption and bone components in blood or urine. Bone metabolism biochemical marks are correlated with osteophorotic agents and also represent significantly different between bone mineral density and bone biochemical marks. Therefore if we develope and use bone metabolism marks which have higher sensitivity and specificity in bone formation and bone absorption, I think that these bone biochemical marks can have utility in the clinical application to predict osteoporosis risk group, bone loss, bone fracture and response degree to treatment of osteoporosis risk groups.

• BMB Reports
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• v.43 no.9
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• pp.579-583
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• 2010

The adipocyte-derived hormone leptin regulates appetite and bone mass. Recent research demonstrates that reciprocally, osteoblasts have a role in controlling energy metabolism. Several genes expressed in osteoblasts are involved in this process, and one of them is the Esp gene. The remaining genes regulate Esp gene expression. OST-PTP, the protein name of Esp, regulates the carboxylation of osteocalcin secreted from osteoblasts, thus affecting insulin sensitivity and insulin secretion. This review provides evidence for a novel interpretation of the connection between bone and energy metabolism and expands our understanding of the novel physiology of bone beyond its classical functions.

• BMB Reports
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• v.46 no.10
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• pp.479-483
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• 2013

The balance between osteoblast-dependent bone formation and osteoclast-dependent bone resorption maintains bone homeostasis. In inflammatory conditions, this balance shifts toward bone resorption, causing osteolytic bone lesions observed in rheumatoid arthritis and periodontitis. A recently discovered family of cytokine IL-17 is widely reported to mediate diverse inflammatory processes. During the last decade, novel roles for IL-17 in skeletal homeostasis have been discovered indicating the potential importance of this cytokine in bone metabolism. This review will summarize and discuss the involvement of IL-17 during bone homeostasis in both physiologic and pathologic conditions. A better understanding of the role of IL-17 in skeletal systems warrants an advance in bone biology, as well as development of therapeutic strategies against bone-lytic diseases, such as rheumatoid arthritis and periodontitis.

• Journal of Nutrition and Health
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• v.31 no.6
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• pp.1039-1048
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• 1998

It has been reported that boron may be beneficial for optimal calcium metabolism and, thus, optimal bone metabolism. Therefore, we designed a study to determine the effect of boron supplementation on Ca and bone metabolism in rats. The rats of 80-l40g body weight were given a control(0ug), 5$\mu\textrm{g}$, 10$\mu\textrm{g}$, 20$\mu\textrm{g}$, 40$\mu\textrm{g}$, or 80$\mu\textrm{g}$ boron supplement per Is diet for 4-weeks. The results are summarized as follows. There were no differences in total food intake and weight gain among the experimental groups. fecal Ca excretion, urinary Ca excretion, apparent Ca absorption, Ca retention, serum alkaline phosphatase activity, and urinary hydroxyproline were not affected by boron supplementation. There was no difference in serum creatinine. Whereas, urinary creatinine excretion was increased with increasing boron supplementation, and conse-quently creatinine clearance was increased with boron supplementation. No differences were found in length, weight, density, Ca content of femur and scapular. The findings suggest that boron supplementation was not effective in Ca and bone metabolism in growing rats fed normal Ca diet. (Korean J Nutrition 31(6) : 1039-1048, 1998)

• 한국유가공학회:학술대회논문집
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• 2007.09a
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• pp.39-47
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• 2007

Bone undergoes continuous remodeling throughout the life and bone health is governed by the balance of bone resorbing osteoclast and bone forming osteoblast. Bone resorption is reflected in tartrate resistant acid phosphatase, pyridinium cross link and collagen telopeptide, whereas bone formation activity can be expressed as bone specific alkaline phosphatase, osteocalcin and procollagen I extension peptide. Milk basic protein and lactoferrin have been reported as active proteins to modulating bone metabolism. In addition to these proteins, some bioactive milk peptides released during lactic fermentation may provide beneficial effect on bone metabolism. The effects of fermented products of Lactobacillus casei ATCC 393 on bone metabolism were investigated using a variety of biochemical markers in osteoblastic MC3T3-E1 cells and ovariectomized rats. Based on the results, the fermented products of Lactobacillus casei ATCC 393 played an functional role in bone metabolism by suppressing bone resorption and by increasing bone formation.

• Proceedings of the Korean Society of Toxicology Conference
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• 2003.05a
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• pp.66-67
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• 2003

It is well known that osteoblasts and osteoc1asts playa key role in bone metabolism. They involve in osteoformation or bone destruction which are ragulated by various factors such as thyroid hormone, parathyroid hormone, estrogen, growth factor and cytokine. Recently, it is demonstrated that oxidative stress is one of pathological factors in bone metabolism, but it is left unknown about mechanism between oxidative stress and bone metabolism.(omitted)

• BMB Reports
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• v.35 no.6
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• pp.609-614
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• 2002

The remodeling process of bone is accompanied by complex changes in the expression levels of various genes. Several approaches have been employed to detect differentially-expressed genes in regard to osteoclast differentiation. In order to identify the genes that are involved in osteoclast differentiation, we used a cDNA-array-nylon membrane. Among 1,200 genes that showed ameasurable signal, 19 genes were chosen for further study. Eleven genes were up-regulated; eight genes were down-regulated. TIS21 was one of the up-regulated genes which were highly expressed in mature osteoclasts. To verify the cDNA microarray results, we carried out RT-PCR and real-time RT-PCR for the TIS21 gene. The TIS21 mRNA level was higher in differentiated-osteoclasts when compared to undifferentiated bone-marrow macrophages. Furthermore, the treatment with $1\;{\mu}M$ of a TIS21 antisense oligonucleotide reduced the formation of osteoclasts from the bone-marrow-precursor cells by ~30%. These results provide evidence for the potential role of TIS21 in the differentiation of osteoclasts.

• Journal of Nutrition and Health
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• v.30 no.7
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• pp.813-824
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• 1997

This study was designed to investigate the effect of dietary calcium and phosphate levels on calcium and bone metabolism in rats. The rats were divided into six groups and each of the groups was fed diets with different Ca/P ratios. The experimental periods were 5 weeks . There was no significant different difference in dietary intake, body weight gain, and organ weight among the groups with different calcium and phosphate intake levels. Fecal calcium excretion was not significantly different among the groups, but urinary calcium excretion was increased by the increase in Ca/P ratio. Fecal phosphate excretion was not different but urinary phosphate excretion was increased by the increase in dietary phosphate intake. There was no significant difference in serum alkaline phophatase activity and urinary hydroxyproline levels were not significantly different among the groups. The low calcium-high phosphate(0.25Ca-1.2% P) group showed the lowest total calcium content in femur and scapula. This may be due to it having the lowest Ca/P ratio among groups. The low calcium-high phosphate(0.2%Ca-1.2%P) group showed that mandible is almost lost and osteolyzed Harversian canal was expanded in femur. Results suggest that phosphate intake affects calcium and bone metabolism more with inadequate calcium nutrition that with adequate calcium intake. Thus , for normal bone growth and metabolism , adequate calcium intake and/or high Ca/P ratio are important.