Runx2 is an essential transcription factor for skeletal development. It is expressed in multipotent mesenchymal cells, osteoblast-lineage cells, and chondrocytes. Runx2 plays a major role in chondrocyte maturation, and Runx3 is partly involved. Runx2 regulates chondrocyte proliferation by directly regulating Ihh expression. It also determines whether chondrocytes become those that form transient cartilage or permanent cartilage, and functions in the pathogenesis of osteoarthritis. Runx2 is essential for osteoblast differentiation and is required for the proliferation of osteoprogenitors. Ihh is required for Runx2 expression in osteoprogenitors, and hedgehog signaling and Runx2 induce the differentiation of osteoprogenitors to preosteoblasts in endochondral bone. Runx2 induces Sp7 expression, and Runx2, Sp7, and canonical Wnt signaling are required for the differentiation of preosteoblasts to immature osteoblasts. It also induces the proliferation of osteoprogenitors by directly regulating the expression of Fgfr2 and Fgfr3. Furthermore, Runx2 induces the proliferation of mesenchymal cells and their commitment into osteoblast-lineage cells through the induction of hedgehog (Gli1, Ptch1, Ihh), Fgf (Fgfr2, Fgfr3), Wnt (Tcf7, Wnt10b), and Pthlh (Pth1r) signaling pathway gene expression in calvaria, and more than a half-dosage of Runx2 is required for their expression. This is a major cause of cleidocranial dysplasia, which is caused by heterozygous mutation of RUNX2. Cbfb, which is a co-transcription factor that forms a heterodimer with Runx2, enhances DNA binding of Runx2 and stabilizes Runx2 protein by inhibiting its ubiquitination. Thus, Runx2/Cbfb regulates the proliferation and differentiation of chondrocytes and osteoblast-lineage cells by activating multiple signaling pathways and via their reciprocal regulation.
A simplified method for the local blood flow determination by means of $^{86}Rb$ was developed in rats and rabbits. $^{86}Rb$ in the form of chloride mixed with physiological saline was intravenously injected. The doses were $10{\mu}Ci$ for rats and $100{\mu}Ci$ for rabbits, which were injected in less than 5 seconds. The rats were sacrificed after 30 seconds, and the rabbits at the intervals of 10, 20, 40 and 60 seconds, by decapitation or rapid intravenous injection of 3 to 5ml of saturated KCI. After bleeding, the organ and tissue samples, e.g. lungs, renal cortex, jejunum and skeletal muscle were quickly removed. The $^{86}Rb$ uptake in 1 gram of the organs and tissues were measured. On the basis of uptake value, administered dose and body weight, the local blood flow was calculated. Following were the results: 1. The uptake values of $^{86}Rb$ in the above organs and tissues of rats were different from other previous reports, in which the large rats were used. It appears, therefore, that the correction on the basis of body weight is necessary. 2. The uptakes of $^{86}Rb$ in the above organs and tissues of rabbits remained rather stationary within 20 to 40 seconds. 3. The local blood flow in the above organs and tissues were calculated from $^{86}Rb$ uptake in per cent dose per 1 gram tissue for 200 gram body weight. The formula could be applied not only to the rabbits but to the rats. 4. The present method could be applied to the comparison of the local blood flow between the various organs and tissues of the control and experimental animals.
The carbonate flat pebble conglomerates (CFPC) are interbedded as lenticular bed in the greenish rhythmite of the upper part of $Hwaj{\check{o}}l$ Formation, $Jos{\check{o}}n$ Supergroup. Pebbles are composed mainly of lime-mudstone with small amounts of bioclasts and silt-sized subangular quartz grains. The matrix among pebbles is composed mainly of sparry calcite with relatively much amounts of bioclasts, silt-sized subangular quartz grains and authigenic pyrite crystals or grains. The sparry calcite of the matrix seems to be the results of neomorphism of skeletal sands and bioclasts. The pebbles are well rounded and no plastic deformations are found. Some pebbles show the outer rim of glauconite. CFPC are not associated with any other intertidal features such as stromatolites, flaser bedding and channel structures. Also any features indicative of subaerial exposure such as dessication cracks, fenestrae and so on are not found in the bed. The sedimentological features of CFPC suggest that the following conditions appear to have been necessary for the formation of CFPC : 1) episodic deposition of thin, permeable calcareous beds separated argillaceous beds; 2) preservation of these beds near the sediment-water interface where they could become rapidly cemented; 3) erosion and redeposition of the partially lithified beds by storms or other exceptional erosional events. Eventually storm erosion and redeposition together represent only one of several critical conditions in the genesis of CFPC. The CFPC are very common in Cambrian and lower Ordovician formations, and become very rare in the younger carbonate formations. The expansion of infauna after Ordovician Period eliminated the widespread potential for rapid submarine cementation which is one of the critical factors to form CFPC.
Oh, Yun Kyo;Choi, Koung Eun;Shin, Youn-Jeong;Kim, Eun Ryoung;Kim, Ji Yeon;Kim, Min Sun;Cho, Sung Yoon;Jin, Dong Kyu
Neonatal Medicine
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v.28
no.3
/
pp.133-138
/
2021
Osteopetrosis refers to a group of genetic skeletal disorders characterized by osteosclerosis and fragile bones. Osteopetrosis can be classified into autosomal dominant, autosomal recessive, or X-linked forms, which might differ in clinical characteristics and disease severity. Autosomal recessive osteopetrosis, also known as malignant osteopetrosis, has an earlier onset, more serious clinical symptoms, and is usually fatal. We encountered a 1-day-old girl who was born full-term via vaginal delivery, which was complicated by meconium-stained amniotic fluid, cephalo-pelvic disproportion, and nuchal cord. Routine neonatal care was provided, in addition to blood tests and chest radiography to screen for sepsis, as well as skull radiography to rule out head injuries. Initial blood tests revealed hypocalcemia, which persisted on follow-up tests the next day. Radiographic examinations revealed diffusely increased bone density and a "space alien" appearance of the skull. Based on radiographic and laboratory findings, the infantile form of osteopetrosis was suspected and genetic testing for identification of the responsible gene. Eventually, a heterozygous mutation of the T cell immune regulator 1, ATPase H+ transporting V0 subunit a3 (TCIRG1) gene (c.292C>T) was identified, making this the first reported case of neonatal-onset malignant osteopetrosis with TCIRG1 mutation in South Korea. Early-onset hypocalcemia is common and usually results from prematurity, fetal growth restriction, maternal diabetes, perinatal asphyxia, and physiologic hypoparathyroidism. However, if hypocalcemia persists, we recommend considering 'infantile of osteopetrosis' as a rare cause of neonatal hypocalcemia and performing radiographic examinations to establish the diagnosis.
Kim, Seung Min;Kim, Cheol Keun;Kim, Soon Heum;Lee, Myung Chul;Kim, Jee Nam;Choi, Hyun Gon;Shin, Dong Hyeok;Jo, Dong In
Archives of Craniofacial Surgery
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v.20
no.3
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pp.186-190
/
2019
Myxomas can be divided into two groups: those derived from the facial skeleton, and those derived from external skeletal soft tissue. Soft tissue myxomas of the head and neck are uncommon, with fewer than 50 cases reported. In any form and location, myxoma of parotid gland is rare. We report a case of myxoma arising from the left superficial lobe of the parotid gland with good long-term follow-up after superficial parotidectomy with tumor excision. A 49-year-old man was referred to our department of plastic and reconstructive surgery with a painless palpable mass that had persisted in the left mandible angle region for 2 years. Excision of the facial mass and superficial parotidectomy with facial nerve preservation were performed. The biopsy result was myxoma. Long-term follow-up for 22 months showed favorable results without evidence of recurrence but with temporary facial nerve weakness right after the surgery. Myxoma should be considered as a differential diagnosis when benign tumor of the parotid gland is being considered.
Ha, Dong Jun;Park, Ji Sun;Jang, Woori;Jung, Na-young;Kim, Su Jin;Moon, Yeonsook;Lee, Jieun
Journal of Genetic Medicine
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v.18
no.2
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pp.110-116
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2021
Microdeletions of chromosome 22q11.2 are one of the most common microdeletions occurring in humans, and is known to be associated with a wide range of highly variable features. These deletions occur within a cluster of low copy repeats (LCRs) in 22q11.2, referred to as LCR22 A-H. DiGeorge (DGS)/velocardiofacial syndrome is the most prevalent form of a 22q11.2 deletions, caused by mainly proximal deletions between LCR22 A and D. As deletions of distal portion to the DGS deleted regions has been extensively studied, the recurrent distal 22q11.2 microdeletions distinct from DGS has been suggested as several clinical entities according to the various in size and position of the deletions on LCRs. We report a case of long-term follow-up of a female diagnosed with a 22q11.2 distal deletion syndrome, identified a deletion of 1.9 Mb at 22q11.21q11.23 (chr22: 21,798,906-23,653,963) using single nucleotide polymorphism array. This region was categorized as distal deletion type of 22q11.2, involving LCR22 D-F. She was born as a preterm, low birth weight to healthy non-consanguineous Korean parents. She showed developmental delay, growth retardation, dysmorphic facial features, and mild skeletal deformities. The patient underwent a growth hormone administration due to growth impairment without catch-up growth. While a height gain was noted, she had become overweight and was subsequently diagnosed with pre-diabetes. Our case could help broaden the genetic and clinical spectrum of 22q11.2 distal deletions.
Journal of the korean academy of Pediatric Dentistry
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v.50
no.4
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pp.483-494
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2023
This report presents two cases concerning strategies for improving denture retention in pediatric patients with oligodontia caused by hypohidrotic ectodermal dysplasia (HED). Both patients presented with multiple missing teeth, conical canines, alveolar bone atrophy, and a skeletal Class III tendency. In the first case, a modified form of conical-crown-retained denture was used to cover the canines. This approach was carried out without tooth extraction or coping. In the second case of severe alveolar bone resorption and a distally tilted lower left canine, the tooth was restored with a hybrid ceramic crown. It was subsequently converted into a clasp-retained removable partial denture, utilizing a suction mechanism. Both patients are currently receiving regular check-ups for the maintenance of their prosthetic appliances and the evaluation of their growth patterns. This study presents innovative prosthetic treatment methods for pediatric patients with HED who have inadequate denture retention.
Kim, Jung Suk;Kim, Cheol Soon;Cha, Jung Yul;Kim, Hee Jin;Hwang, Chung Ju
Journal of the Korean Academy of Esthetic Dentistry
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v.24
no.1
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pp.4-12
/
2015
Purpose: Due to the presence of various muscles around lips, variety of facial expression can be made and changes from aging process such as wrinkles can develop on the facial skin by the action of multiple muscles. In animals, skin and muscles are developed in the entire body. On contrast, they are well developed only in the face and just one is present in the neck and the palm. Alar band was defined as outer wrinkle formed by zygomaticus minor muscle, which is common in Koreans. This study aimed to investigate clinical prevalence of alar band. Materials & Methods: Subjects were chosen from 780 new patients who visited private clinic in Gyeonggi province for orthodontic treatment. Presence of alar band was examined from the smile extraoral photos. Correlation among skeletal form, lip protrusion, gender, and age were evaluated. Results: Prevalence of alar band was higher in women (27.9%) than in men (18.5%) with statistical significance (p<0.05). With respect to age, prevalence of alar band was 19.4% in age 0-9 y, 16.9% in age 10-19 y, 31.2% in age 20-29 y, 39.5% in age 30-39, 56.5% in age 40-49. Prevalence was gradually increased from patients in their 20s to patients in their 40s and statistical significance was found (p<0.001). Concerning SN_NP, prevalence was 26.2% in normodivergent facial type, 22.0% in hyperdivergent facial type, and 32.2% in hypodivergent facial type. Hypodivergent facial group had higher prevalence but statistical significance was not observed. Statistically significant difference was not found regarding upper lip. However, prevalence of the alar band was 26% in patients with normal lower lip, 14.7% in patients with pretruded lower lip, and 33.3% in retruded lower lip. The prevalence was higher in patients with retruded lower lip with statistical significance (p<0.05). Conclusions: 27.8% on previous anatomical study and this study showed 27.8% prevalence of alar band in clinical smile photographs. Clinical photograph study showed that alar band was more prominent in women, older people, and people with retruded lips with statistical significance. This will provide valuable diagnostic information for esthetic consideration.
The anatomical structure of the Skeleton of thoracic limb of thirty-one adult Korean native goats(body weight: 14~17kg) was observed after skeletal preparation, and the osteometry was performed in each bone. The results were as follows; 1. The thoracic limb of the Korean native goat was composed of scapula, humerus, radius, ulna, carpal bones, metacarpal bones, phalanges and sesamoid bones. 2. The scapula was flat and triangular in shape. There were no distinct tuber of spine and acromion in the spine. The subscapular fossa was deep and triangular in shape and the vertebral border was sigmoid form. The coracoid bone was formed as the coracoid process at the medial aspect of the supraglenoid tubercle but the clavicle wa.s not observed. The left and right scapular indexes were 57.92 and 58.31 and the glenoid cavity indexes were 89.23 and 86.82, respectively. 3. The greater tubercle of the humerus was devided into cranial and caudal parts. The third tubercle was observed and the face for the infraspinatus muscle was rectangular form. The left and right humerus indexes were 32.44 and 32.63, the head indexes were 94.13, 96.62 and the trochlear-epidondyle indexes were 67.32 and 65.81, respectively. 4. The radius and ulna were fused entirely except at the broad proximal and narrow distal interosseous spaces. The ulna was longer than the radius, and its reduced body and distal end were fused at the caudomedial surface of the radius. 5. The carpal bones were six in number. There were radial, intermediate, ulnar, accessory, second-third and fourth carpal hones in carpal bones. 6. The metacarpal bone was composed of a large metacarpal bone resulted from the fusion of the third and fourth metacarpal bones, and there was a metacarpal tubercle at the dorsolateral part of the proximal end. There were no vestiges of the second and fifth metacarpal bones. 7. The digits were composed of third and fourth digits and each digit was composed of the proximal, middle and distal phalanges. 8. The sesamoid bones were six in number. There were two at the fetlock joint and one at the coffine joint palmarly in each digit. 9. The ratios of the lengths among the scapula, humerus, antebrachium and metacarpal bone were 1.42 : 1.47 : 1.77 : 1.00 in the left and 1.42 : 1.45 : 1.77 : 1.00 in the right, respectively.
Kim, Jin-sang;Lee, Heung-shik S.;Lee, In-se;Yoon, Yeo-sung
Korean Journal of Veterinary Research
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v.28
no.1
/
pp.1-16
/
1988
The anatomical structure of pelvic limb, of thirty-one adult Korean native goats (Body weight: 14~17kg) was observed after skeletal preparation, and the osteometry was performed in each bone. The results were as follows: 1. The pelvic limb of the Korean native goat was composed of the hip bone, femur, patella, tibia, fibula, tarsal bones, metatarsal bone, phalanges and sesamoid bones. 2. The hip bone consisted of the ilium, ischium and pubis which fused each other, The gluteal surface of the ilium was directed dorsolaterally. The tuber sacrale and tuber coxae were formed at the dorsal apex and ventrolateral part of the iliac wing, respectively. The lesser ischiatic notch was deeper than the greater one. The ischiatic tubercles were triangular form and consisted of the dorsal, lateral and caudal ischiatic tubercles. The left and right hip bone indexes were 67.08, 66.20, the acetabular indexes were 93.78 and 92.10 and the obturator foramen. indexes were 53.84 and 54.77, respectively. 3. In femur, both of the greater and lesser trochanter were well developed but the third trochanter was not observed. The left and right femur indexes were 26.55 and 26.14, head indexes were 81.66 and 81.49 and the trochlear-epicondyle indexes were 42.47 and 41.63, respectively. 4. The patella was observed as an isosceles triangle with base lying proximal and the cranial surface was more convex. 5. The tibial shaft was sigmoid form and the popliteal notch was deep. There was a large nutrient foramen at the cranial aspect of the cranial intercondylar area. The tibial indexes were 22.09 in left and 21.10 in right. 6. The proximal extremity of the fibula was fused with the lateral condyle of the tibia but the distal one was observed independently as the malleolar bone. 7. The tarsal bones were five in number; the talus, calcaneus, centroquartal tarsal bone, first tarsal bone, and second-third tarsal bone. 8. The metatarsal bone was composed of a large metatarsal bone. resulted from the fusion of the third and fourth metatarsal bones, The structure of metatarsal bone was similar to the metacarpal bone but longer about 7mm. 9. The phalanges and sesamoid bones were similar to these of the thoratic limb. 10. The ratios of the lengths among the hip bone, femur, tibia and metafarsal bone were 1.71 : 1.54 : 1.73 : 1.00 in left and 1.68 : 1.53 : 1.72 : 1.00 in right, respectively.
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