• Food Science of Animal Resources
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• v.43 no.2
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• pp.359-373
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• 2023

This study examined the α-glucosidase inhibitory, and apoptosis- and anti-muscular-related factors of goat meat extracts from forelegs, hind legs, loin, and ribs. The goat meat extracts were evaluated for their α-glucosidase inhibitory activity. The gene and protein expression levels of Bcl-2-associated X (bax), p53, and p21 were examined by reverse transcription polymerase chain reaction (RT-PCR) and immunoblotting in AGS and HT-29 cells. The expression levels of Atrogin-1 and MHC1b were examined by RT-PCR in C2C12 myoblasts, and the expression levels of Atrogin-1, muscle atrophy F-box (MAFbx), muscle RING-finger protein-1 (MuRF-1), and myosin heavy chain-7 were investigated by immunoblotting. α-Glucosidase inhibitory activity was higher in ethanol extract than in hydrous and hot water extracts. BAX and p53 expression levels were higher (p<0.05) in AGS cells treated with goat meat extract than those of cells treated with no goat meat extract. In HT-29 cells, the protein expression levels of BAX, p53, and p21 were higher (p<0.05) in the cells treated with goat meat extract than those of cells not treated with goat meat extract. In dexamethasone-treated C2C12 cells, goat meat extract treatment lower (p<0.05) the expression of Atrogin-1 and lower (p<0.05) the expression of MAFbx and MuRF-1. The results of the present study indicate that goat meat extracts have α-glucosidase inhibitory activity in vitro. In addition, apoptosis was induced in AGS cells and HT-29 cells treated with goat meat extract, and anti-muscular atrophy activity was also observed in C2C12 cells treated with goat meat extract.

• Journal of Genetic Medicine
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• v.1 no.1
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• pp.33-37
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• 1997

Spinal muscular atrophy (SMA) is the second most common fatal disease of childhood with autosomal dominant mode of inheritance, and in its less severe form the third most common neuromuscular disease of childhood after Duchenne muscular dystrophy. The genetic defect was found to be on the long arm of chromosome 5 (5q11.2-q13.3) where many genes and microsatellite markers were missing. One of the most important genes is the Survival Motor Neuron (SMN) gene which is homozygously missing in 90% of SMA patients. Another important gene, the Neuronal Apoptosis Inhibitory Protein (NAIP) gene was found to be defective in 67% of SMA type I patients. Studies so far suggest SMA occurs when the genes on the long arm of chromosome 5 are mutated or deleted. Recently our hospital encountered 2 SMA patients of type I and II respectively. These patients both had homozygously defective SMN genes but intact NAIP genes. We are reporting these cases with bibliographic review and discussion. Korean SMA patients presumably have defects in SMN genes similar to those found in European patients, although the significance of NAIP genes remains to be established. SMN gene defects can be easily diagnosed using PCR and restriction enzymes, and this method could be applied towards convenient prenatal diagnosis and towards screening for family members at risk.

• Annals of Clinical Neurophysiology
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• v.17 no.1
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• pp.1-16
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• 2015

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that is characterized by progressive death of motor neurons in the cortex, brainstem, and spinal cord. Until now, many treatment strategies have been tested in ALS, but so far only Riluzole has shown efficacy of slightly slowing disease progression. The pathophysiological mechanisms underlying ALS are multifactorial, with a complex interaction between genetic factors and molecular pathways. Other motor neuron disease such as spinal muscular atrophy (SMA) and spinobulbar muscular atrophy (SBMA) are also progressive neurodegenerative disease with loss of motor neuron as ALS. This common thread of motor neuron loss has provided a target for the development of therapies for these motor neuron diseases. A better understanding of these pathogenic mechanisms and the potential pathological relationship between the various cellular processes have suggested novel therapeutic approaches, including stem cell and genetics-based strategies, providing hope for feasible treatment of ALS.

• Journal of the Korean Society of Physical Medicine
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• v.16 no.2
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• pp.101-113
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• 2021

PURPOSE: The purpose of this study was to investigate the differences and correlation between body composition, exercise performance, and behavior based on playing football in childhood. METHODS: 16 subjects who played football in childhood participated in the study. Body composition and exercise performance were measured, and problem behavior was assessed for each of them. All subjects were asked to play football 50 min/day, one day/week for 8-weeks. RESULTS: Muscle mass, muscular strength, balance, and cardiopulmonary endurance, anxiety depression, atrophy depression, attention problems, rule violations, DSM somatization problems, DSM rebellious behavior problems, and sociality significantly increased after 8-weeks. There was a negative (-) correlation between anxiety depression and atrophy depression, and DSM somatization problem and muscular strength, attention problem and balance, and rule violation and cardiopulmonary endurance, after playing football. CONCLUSION: These results confirmed that playing football in childhood had a positive effect on body composition, and that exercise performance and problem behavior were related.

• Clinical and Experimental Pediatrics
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• v.53 no.11
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• pp.965-970
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• 2010

Purpose: The life expectancy of patients with spinal muscular atrophy (SMA) type I is generally considered to be less than 2 years. Recently, with the introduction of proactive treatments, a longer survival and an improved survival rate have been reported. In this study, we analyzed the natural courses and survival statistics of SMA type I patients and compared the clinical characteristics of the patients based on their survival periods. Methods: We reviewed the medical records of 14 pediatric patients diagnosed with SMA type I during a 9-year period. We examined the demographic and clinical characteristics of these patients, calculated their survival probabilities, and plotted survival curves as on the censoring date, January 1, 2010. We also compared the characteristics of the patients who died before the age of 24 months (early-death, ED group) and those who survived for 24 months or longer (long-survival, LS group). Results: The mean survival time was $22.8{\pm}2.0$ months. The survival probabilities at 6 months, 12 months, 18 months, 24 months, and 30 months were 92.9%, 92.9%, 76.0%, 76.0%, and 65.1%, respectively. Birth weight was the only factor that showed a statistically significant difference between the ED and LS groups ($P$=0.048). Conclusion: In this study, the survival probabilities at 2 years were far greater than expected. Because of the limited number of patients and information in this study, the contribution of improved supportive care on longer survival could not be clarified; this may be elucidated in larger cohort studies.

• Clinical and Experimental Pediatrics
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• v.51 no.12
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• pp.1350-1354
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• 2008

Spinal muscular atrophy (SMA) is an autosomal recessive disease characterized by diffuse proximal and distal weakness due to deletion of the survival motor neuron (SMN) gene localized on chromosome 5 (5q11.2-13.3). SMA has been considered as a pure lower motor neuron disorder, and a definitive diagnosis can be established by molecular genetic testing. Here, we describe two patients with severe hypotonia and frequent aspirations at early infancy. Nerve conduction studies showed more extensive sensory involvement in these patients diagnosed to have SMA by genetic study than in classical cases of SMA. To the best of our knowledge, this is the first report of SMA Type 1 with sensory nerve involvement in Korea.

• Clinics in Shoulder and Elbow
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• v.22 no.2
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• pp.70-78
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• 2019

Background: This study evaluated postoperative changes in the supraspinatus from time-zero to 6 months, using magnetic resonance imaging (MRI). We hypothesized that restoration of the musculotendinous unit of the rotator cuff by tendon repair immediately improves the rotator cuff muscle status, and maintains it months after surgery. Methods: Totally, 76 patients (29 men, 47 women) with rotator cuff tears involving the supraspinatus tendon who underwent arthroscopic rotator cuff repairs were examined. MRI evaluation showed complete repair with intact integrity of the torn tendon at both time-zero and at 6 months follow-up. All patients underwent standardized MRI at our institution preoperatively, at 1 or 2 days postoperative, and at 6 months after surgery. Supraspinatus muscular (SSP) atrophy (Thomazeau grade) and fatty infiltrations (Goutallier stage) were evaluated by MRI. The cross-sectional area of SSP in the fossa was also measured. Results: As determined by MRI, the cross-sectional area of SSP significantly decreased 11.41% from time-zero (immediate repair) to 6 months post-surgery, whereas the Goutallier stage and Thomazeau grade showed no significant changes (p<0.01). Furthermore, compared to the preoperative MRI, the postoperative MRI at 6 months showed a no statistically significant increase of 8.03% in the cross-sectional area. In addition, morphological improvements were observed in patients with high grade Goutallier and Thomazeau at time-zero, whereas morphology of patients with low grade factors were almost similar to before surgery. Conclusions: Our results indicate that cross-sectional area of the initial repair appears to decrease after a few months postoperatively, possibly due to medial retraction or strained muscle.

• Journal of rehabilitation welfare engineering & assistive technology
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• v.10 no.2
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• pp.107-112
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• 2016

The purpose of this study was to determine the effect of aquatic ramp walking exercise on the activity of the quadriceps, gait and activity of daily living in child with Spinal Muscular Atrophy (SMA) type II. A 5 years-old girl with SMA type II participated in this study. This study used single-subject reverse(A-B-A) design study. There are 12 sessions(4weeks 3 times a week) each during the baseline phase(A), the intervention phase(B), the follow up phase(A). During the baseline phase and the follow up phase performed general aquatic therapy, the intervention phase additional performed walking activity on ramp in pool (60m). Surface electromyogram, Timed Up and Go (TUG) test, ACTIVLIM were used as outcome. During the intervention phase, there were decrease on the activity of the quadriceps. In modified TUG test, gait time reduced during the intervention phase. The ACTIVLIM logit score increased during intervention phase by comparison with the baseline phase. These findings suggest that an aquatic ramp walking exercise activities have the therapeutic possibility on the quadriceps activity and gait ability for child with SMA type II.

• Journal of Life Science
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• v.28 no.4
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• pp.435-443
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• 2018

AMP-activated protein kinase (AMPK) functions as a metabolic master through regulating and restoring cellular energy balance. In skeletal muscle, AMPK increases myofibril protein degradation through the expression of muscle-specific ubiquitin ligases. Mori Folium, the leaf of Morus alba, is a traditional medicinal herb with various pharmacological functions; however, the effects associated with muscle atrophy have not been fully identified. In this study, we confirmed the effects of AMPK activation by examining the effects of 5-aminoimidazole-4-carboxamide ribonucleotide (AICAR), an activator of AMPK, on the induction of atrophy and expression of atrophy-related genes in C2C12 myotubes. We also investigated the effects of the ethanol extract of Mori Folium (EEMF) on the recovery of AICAR-induced muscle atrophy in C2C12 myotubes. It was found that exposure to AICAR resulted in the stimulation of Forkhead box O3a (FOXO3a); an up-regulation of muscle-specific ubiquitin ligases such as Muscle Atrophy F-box (MAFbx)/atrogin-1 and muscle RING finger-1 (MuRF1), and a down-regulation of muscle-specific transcription factors, such as MyoD and myogenin; with the activation of AMPK. In addition, AICAR without cytotoxicity indicated a decrease in diameter of C2C12 myotubes. However, treatment with EEMF significantly suppressed AICAR-induced muscle atrophy of C2C12 myotubes in a dose-dependent manner as confirmed by a decrease in myotube diameter, which is associated with a reversed stimulation of FOXO3a by the inhibition of AMPK activation. These results indicate that the activation of AMPK by AICAR induces muscle atrophy, and EEMF has preeminent effects on the inhibition of AICAR-induced muscle atrophy through the AMPK signaling pathway.

• Journal of Korean Academy of Nursing
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• v.43 no.3
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• pp.361-369
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• 2013

Purpose: The purpose of this study was to examine the effect of anorexia and neuropathic pain induced by cisplatin on hindlimb muscles of rats. Methods: Adult male Sprague-Dawley rats were divided into two groups, a cisplatin-treated group (n=10) and a control group (n=10). In the cisplatin-treated group, cisplatin at a dose of 2 mg/kg was injected intraperitoneally two times a week up to a cumulative dose of 20 mg/kg over 5 weeks, and in the control group saline (0.9% NaCl) was injected intraperitoneally at the same dose and duration as the cisplatin-treated group. At 34 days all rats were anesthetized, after which the soleus and plantaris muscles were dissected. Withdrawal threshold, body weight, food intake, activity, muscle weight, Type I and II fiber cross-sectional areas and myofibrillar protein content of the dissected muscles were determined. Results: Compared with the control group, the cisplatin-treated group showed significant decreases (p<.05) in withdrawal threshold, activity, food intake, body weight, Type I and II fiber cross-sectional areas, myofibrillar protein content and weight of the soleus and plantaris muscles. Conclusion: Muscular atrophy in hindlimb occurs due to anorexia and neuropathic pain induced by the cisplatin treatment.