Congenital hemihypertrophy is a rare idiopathic condition, first described by Meckel in 1822 and represents enlargement of a part or entire side of the body without associated vascular or neurologic disease. Wagner recorded the first case in the 1839. Hemihypertrophy is to be differentiated from hemiatrophy (which involves unilateral subnormal development, muscle weakness, or neurologic deficit) and the syndromes of hemidystrophy. Approximately 25 - 50% of the reported cases of hemihypertrophy have been associated with hamartomas or congenital defects, especially genitourinary anomalies. The early detection of asymptomatic intraabdominal tumor in patients with congenital hemihypertrophy is important We report 3 cases of congenital hemihypertrophy with discussion of the clinical manifestations and associated anomalies, which were rarely reported in domestic literatures.
Congenital Abnormalities ; Hamartoma ; Humans ; Muscle Development

The growth and development of skeletal muscle is an important factor affecting pork production and quality, which is elaborately regulated by many genetic and nutritional factors. MicroRNA (miRNA) is a non-coding RNA with a length of about 22 nt, which binds to the 3'UTR sequence of the mRNA of the target genes, and consequently regulates its post-transcriptional expression level. In recent years, a large number of studies have shown that miRNAs are involved in various life processes such as growth and development, reproduction, and diseases. The role of miRNAs in the regulation of porcine skeletal muscle development was reviewed, with the hope to provide a reference for the genetic improvement of pigs.
Swine ; Animals ; MicroRNAs/metabolism* ; Muscle, Skeletal/metabolism* ; Muscle Development/genetics*

PURPOSE: To determine the prevalence of scoliosis in volleyball athletes and compare this with the prevalence in the general population. MATERIALS AND METHODS: One hundred and sixteen volleyball athletes who had been enrolled in the activity for more than one year were examined for prevalence of scoliosis. The Adams forward bending test was performed with a measurement of the truncal asymmetry using a scoliometer (Orthopaedic System, Inc, USA). Those atheletes who showed more than 5degreesof measurement were selected for an X-ray evaluation. Data from a randomized point prevalence survey of Korean Middle school students (46,428) in Seoul City, which had been performed by our department, was adopted for the control group. RESULTS: Among the 116 volleyball players, 60 (51.7%) showed more than a 5degreesof angle of trunk rotation, whereas controls of middle school students showed 2.5%. Cobb's angle more than 10degrees was 6 (5.17%) in athletes and the control group was 465 (1.0%). Despite higher frequency of prevalence, the Cobb' angle was below 15degrees whereas the control group showed a severe scoliosis which of Cobb's angle reaching to 45degrees. CONCLUSION: Volleyball athletes showed a higher incidence of truncal asymmetry and scoliotic spinal columns than the control group. However, we were able to conclude that asymmetrical muscle development can produce a mild scoliosis. However this doesn't have the potential for a severe progression as found in some cases of idiopathic scoliosis.
Athletes* ; Humans ; Incidence ; Muscle Development ; Prevalence ; Scoliosis* ; Seoul ; Spine ; Volleyball*

OBJECTIVE: To evaluate the pattern of developmental delay of language and to correlate the language with other developmental areas in cerebral palsy children. METHOD: Sequenced Inventory of Communication Development (SICD) was studied in 31 children with cerebral palsy of age ranging from 11 months to 48 months. Korean Denver Developmental Screening Test (DDST) was also performed in 18 children simultaneously. RESULTS: On SICD, 10 children (32.3%) showed the receptive language delay and 13 children (41.9%) showed the expressive language delay. Among 15 spastic quadriplegic children, 40% showed the delay of receptive language development, 53.3% showed the delay in expressive language development. Among 10 spastic diplegic children, 30% showed the delay of both receptive and expressive language development. One spastic right hemiplegic child showed a delay of expressive language development, but 4 left hemiplegic children showed the normal language development. One hypotonic cerebral palsy child showed a delay of both receptive and expressive language development. The expressive language was delayed more than the receptive language. SICD correlated highly with the language sector of DDST. And both SICD and DDST language sectors correlated with the other sectors of DDST (personal-social, fine motor-adaptive, gross motor), especially with the fine motor sector (r=0.912, 0.918, 0.976, p<0.001). CONCLUSION: There is a considerably high incidence of developmental delay of language in cerebral palsy children, especially among spastic quadriplegic children. The early evaluation and treatment for the developmental delay of language need to be included in a general rehabilitation program for the cerebral palsy children.
Cerebral Palsy* ; Child* ; Humans ; Incidence ; Language Development ; Language Development Disorders ; Mass Screening ; Muscle Spasticity ; Rehabilitation

Replication-independent incorporation of variant histone H3.3 has a profound impact on chromatin function and numerous cellular processes, including the differentiation of muscle cells. The histone chaperone HIRA and H3.3 have essential roles in MyoD regulation during myoblast differentiation. However, the precise mechanism that determines the onset of H3.3 deposition in response to differentiation signals is unclear. Here we show that HIRA is phosphorylated by Akt kinase, an important signaling modulator in muscle cells. By generating a phosphospecific antibody, we found that a significant amount of HIRA was phosphorylated in myoblasts. The phosphorylation level of HIRA and the occupancy of phosphorylated protein on muscle genes gradually decreased during cellular differentiation. Remarkably, the forced expression of the phosphomimic form of HIRA resulted in reduced H3.3 deposition and suppressed the activation of muscle genes in myotubes. Our data show that HIRA phosphorylation limits the expression of myogenic genes, while the dephosphorylation of HIRA is required for proficient H3.3 deposition and gene activation, demonstrating that the phosphorylation switch is exploited to modulate HIRA/H3.3-mediated muscle gene regulation during myogenesis.
Antibodies, Phospho-Specific ; Chromatin ; Histones* ; Muscle Cells* ; Muscle Development ; Muscle Fibers, Skeletal ; Myoblasts ; Phosphorylation* ; Phosphotransferases ; Transcriptional Activation

Myoblast fusion depends on mitochondrial integrity and intracellular Ca²⁺ signaling regulated by various ion channels. In this study, we investigated the ionic currents associated with [Ca²⁺]i regulation in normal and mitochondrial DNA-depleted (ρ0) L6 myoblasts. The ρ0 myoblasts showed impaired myotube formation. The inwardly rectifying K⁺ current (I(Kir)) was largely decreased with reduced expression of KIR2.1, whereas the voltage-operated Ca²⁺ channel and Ca²⁺-activated K⁺ channel currents were intact. Sustained inhibition of mitochondrial electron transport by antimycin A treatment (24 h) also decreased the I(Kir). The ρ0 myoblasts showed depolarized resting membrane potential and higher basal [Ca²⁺]ᵢ. Our results demonstrated the specific downregulation of I(Kir) by dysfunctional mitochondria. The resultant depolarization and altered Ca²⁺ signaling might be associated with impaired myoblast fusion in ρ0 myoblasts.
Antimycin A ; Down-Regulation ; Electron Transport ; Ion Channels ; Membrane Potentials ; Mitochondria ; Muscle Development ; Muscle Fibers, Skeletal ; Myoblasts* ; Oxidative Phosphorylation*

BCL-2 interacting cell death suppressor (BIS), which is ubiquitously expressed, has important roles in various cellular processes, such as apoptosis, the cellular stress response, migration and invasion and protein quality control. In particular, BIS is highly expressed in skeletal and cardiac muscles, and BIS gene mutations result in human myopathy. In this study, we show that mRNA and protein levels of BIS were markedly increased during skeletal myogenesis in C2C12 cells and mouse satellite cells. BIS knockdown did not prevent the early stage of skeletal myogenesis, but did induce muscle atrophy and a decrease in the diameter of myotubes. BIS knockdown significantly suppressed the expression level of myosin heavy chain (MyHC) without changing the expression levels of myogenic marker proteins, such as Mgn, Cav-3 and MG53. In addition, BIS endogenously interacted with MyHC, and BIS knockdown induced MyHC ubiquitination and degradation. From these data, we conclude that molecular association of MyHC and BIS is necessary for MyHC stabilization in skeletal muscle.
Animals ; Apoptosis ; Cell Death* ; Humans ; Mice ; Muscle Development ; Muscle Fibers, Skeletal ; Muscle, Skeletal* ; Muscular Atrophy ; Muscular Diseases ; Myocardium ; Myosin Heavy Chains* ; Myosins* ; Quality Control ; RNA, Messenger ; Ubiquitin ; Ubiquitination

BCL-2 interacting cell death suppressor (BIS), which is ubiquitously expressed, has important roles in various cellular processes, such as apoptosis, the cellular stress response, migration and invasion and protein quality control. In particular, BIS is highly expressed in skeletal and cardiac muscles, and BIS gene mutations result in human myopathy. In this study, we show that mRNA and protein levels of BIS were markedly increased during skeletal myogenesis in C2C12 cells and mouse satellite cells. BIS knockdown did not prevent the early stage of skeletal myogenesis, but did induce muscle atrophy and a decrease in the diameter of myotubes. BIS knockdown significantly suppressed the expression level of myosin heavy chain (MyHC) without changing the expression levels of myogenic marker proteins, such as Mgn, Cav-3 and MG53. In addition, BIS endogenously interacted with MyHC, and BIS knockdown induced MyHC ubiquitination and degradation. From these data, we conclude that molecular association of MyHC and BIS is necessary for MyHC stabilization in skeletal muscle.
Animals ; Apoptosis ; Cell Death* ; Humans ; Mice ; Muscle Development ; Muscle Fibers, Skeletal ; Muscle, Skeletal* ; Muscular Atrophy ; Muscular Diseases ; Myocardium ; Myosin Heavy Chains* ; Myosins* ; Quality Control ; RNA, Messenger ; Ubiquitin ; Ubiquitination

BACKGROUND: Popeye deformity is common after rupture of the biceps muscle's long head tendon. Herein, we report on histological changes in biceps brachii muscles following tenotomy of the long head biceps tendon. METHODS: Twelve Sprague-Dawley rats (12-week-old) underwent tenotomy of the long head biceps tendon in the right shoulder. At postoperative weeks 4, 7, and 10, the operative shoulders were removed by detaching the biceps brachii muscle from the glenoid scapula and humerus; the opposite shoulders were removed as controls. H&E staining was performed to elucidate histological changes in myocytes. Oil-red O staining was performed to determine fatty infiltration. Myostatin antibody immunohistochemistry staining was performed as myostatin is expressed by skeletal muscle cells during myogenesis. RESULTS: H&E staining results revealed no changes in muscle cell nuclei. There were no adipocytes detected. Compared with that of the control biceps, the cross-sectional area of the long head biceps was significantly smaller (p=0.00). Statistical changes in the total extent of the 100 muscle cells were significant (p=0.00). Oil-red O staining revealed no fatty infiltration. Myostatin antibody immunohistochemical staining revealed no significant difference between the two sides. CONCLUSIONS: Muscular changes after tenotomy of the long head biceps included a decrease in the size of the individual muscle cells and in relative muscle mass. There were no changes observed in muscle cell nuclei and no fatty infiltration. Moreover, there were no changes detected by myostatin antibody immunohistochemistry assay.
Adipocytes ; Animals ; Congenital Abnormalities ; Head ; Humerus ; Immunohistochemistry ; Models, Animal ; Muscle Cells ; Muscle Development ; Muscle, Skeletal ; Muscles ; Myostatin ; Rats ; Rats, Sprague-Dawley ; Rupture ; Scapula ; Shoulder ; Tendons ; Tenotomy

Laminin is an extracellular matrix-associated protein, which is largely present in the basement membranes of the human placenta, striated muscle and Schwann cell. Laminin has been proposed to promote attachment, spread, motility, growth and development of tissues or cells. In this study, we investigated the formation, localization and migration of the laminin in developing rectus femoris muscle of fetal and newborn rats. Experimental animals, fetal or newborn rats (Sprague-Dawley strain), were divided into 8 groups (14 day-old, 16 day-old, 18 day-old, 20 day-old and 22 day-old fetal rats, 1 day-old, 3 day-old, 5 day-old and 7 day-old newborn rats). The specimens of each group were prepared for detection of the laminin by immuno- histological and immunogold electron microscopic methods. 1. The primitive rectus femoris muscle of 14 day-old fetal rats consisted of mesenchymal cells and myoblasts. Laminin stained with a few gold particles were observed in the cytoplasm of myoblasts and rough endoplasmic reticulum of mesenchymal cells. 2. In the rectus femoris muscle of 16 day-old fetal rats, laminin was strongly expressed in myoblasts and mesenchymal cells. Gold particles were distributed in the cytoplasm of myoblasts and mesenchymal cells. 3. In the rectus femoris muscle of 18 and 20 day-old fetal rats, strong laminin immune reactions were observed in the basement membrane of muscle cells. A few gold particles distributed on the basal lamina of muscle cells and in the RER of fibroblasts. 4. In the rectus femoris muscle of 22 day-old fetal rats and 1 day-old newborn rats, strong laminin immune reactions were seen in the basement membrane of muscle cells. Numerous gold particles were located on the basal lamina of muscle cells and the RER of fibroblasts. 5. In the rectus femoris muscle of 3 day, 5 day and 7 day-old newborn rats, moderate laminin immune reactions were detected in the basement membrane of the muscle cell. A few gold particles were located on the basal laminae of muscle cells and RER of fibroblasts. These results suggest that the distribution of laminin in the rectus femoris muscle is related to myogenicity. Laminin seemed to be secreted by myoblasts of rectus femoris muscle at the fetal stage, but by fibroblasts in the muscle at neonatal and adult stages.
Adult ; Animals ; Basement Membrane ; Cytoplasm ; Endoplasmic Reticulum, Rough ; Fibroblasts ; Growth and Development ; Humans ; Infant, Newborn ; Laminin* ; Muscle Cells ; Muscle, Striated ; Myoblasts ; Placenta ; Quadriceps Muscle* ; Rats*