Ubiquitin signaling regulates virtually all aspects of eukaryotic biology and dynamic processes in which protein substrates are modified by ubiquitin. To regulate these processes, deubiquitinating enzymes (DUBs) cleave ubiquitin or ubiquitin-like proteins from these substrates. DUBs have been implicated in the pathogenesis of cancer, leading to the development of increasing numbers of small-molecule DUB inhibitors. On the other hand, recent studies have focused on the function of DUBs in metabolic diseases such as obesity, diabetes, and fatty liver diseases. DUBs play a positive or negative role in the progression and development of metabolic diseases. Their involvement in cell pathology and regulation of major transcription factors in metabolic syndrome has been examined in vitro and in animal and human biopsies. UCH, USP7, and USP19 were linked to adipocyte differentiation, body weight gain, and insulin resistance in genetic or diet-induced obesity. CYLD, USP4, and USP18 were found to be closely associated with fatty liver diseases. In addition, these liver diseases were accompanied by body weight change in certain cases. Collectively, in this review, we discuss the current understanding of DUBs in metabolic diseases with a particular focus on obesity. We also provide basic knowledge and regulatory mechanisms of DUBs and suggest these enzymes as therapeutic targets for metabolic diseases.
Journal of the korean academy of Pediatric Dentistry
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v.29
no.2
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pp.237-242
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2002
Lowe syndrome, also known as oculocerebrorenal syndrome, is a rare genetic disorder involving eyes, kidney and nervous system, and occurs predominantly in mostly males. The patients with Lowe syndrome are characterized with prominent forehead, thin and sparse hair, protruding ears, congenital cataracts, glaucoma, mental retardation, stunted growth, hypotonia, decrease in muscle mass and tendon reflexes, renal tubular dysfunction, and metabolic bone disease. A 6-year-old boy with Lowe syndrome was admitted to our clinic, with multiple caries and a chief complaint of intermittent pain on the left mandibular molar area. Because of difficulty in management of behavior and his medical problem, general anesthesia was performed for dental care. No specific complication was noticed during dental treatment procedure under general anesthesia and also during periodic recall-checks. General anesthesia itself, however, could be a potentially life-threatening procedure due to patient's biomedical problems. When a dental procedure under general anesthesia is to be required in patient with Lowe syndrome, it may be advisable being teamed with physicians, and general anesthesia duration should be as short as possible.
Han, Hyun-Ja;Lee, Nam Sil;Kim, Myoung Sug;Jung, Sung Hee
Fisheries and Aquatic Sciences
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v.18
no.3
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pp.333-339
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2015
Red lip mullet Chelon haematocheilus (body weight = $468{\pm}91g$) which became sick during an outbreak of disease at mariculture facilities at Cheonsu Bay, Korea, during July-August 2013, were examined to identify the cause of the disease. Diseased mullets displayed green liver syndrome, and Lactococcus garvieae were isolated from their internal organs. Argulus sp., Trichodina sp., and/or Vibrio spp. were also discovered in some infected fish. Histopathological examination revealed that fatty liver syndrome with hepatocyte degeneration, reflected in heterokaryons, inflammatory lesions, and melanomacrophage centers ($MMC_S$), had caused fibrosis around the kidney, spleen, and blood vessels. After the outbreak, visceral fat and green liver syndrome in the mullets were consistently observed throughout the year in the same mariculture facilities, indicating that the cultured mullets suffered a chronic metabolic disorder. Although Vibrio spp. were also isolated from some individuals, L. garvieae, which is known to be a causative agent of red lip mullet mortality, was isolated from all diseased individuals. This is the first report of L. garvieae infection in cultured red lip mullet.
Fernandez, Maria Luz;Jones, Jennifer J.;Ackerman, Daniela;Barona, Jacqueline;Calle, Mariana;Comperatore, Michael V.;Kim, Jung-Eun;Andersen, Catherine;Leite, Jose O.;Volek, Jeff S.;McIntosh, Mark;Kalynych, Colleen;Najm, Wadie;Lerman, Robert H.
Nutrition Research and Practice
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v.4
no.6
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pp.492-498
/
2010
Both metabolic syndrome (MetS) and elevated LDL cholesterol (LDL-C) increase the risk for cardiovascular disease (CVD). We hypothesized that low HDL cholesterol (HDL-C) would further increase CVD risk in women having both conditions. To assess this, we recruited 89 women with MetS (25-72 y) and LDL-C ${\geq}$ 2.6 mmol/L. To determine whether plasma HDL-C concentrations were associated with dietary components, circulating atherogenic particles, and other risk factors for CVD, we divided the subjects into two groups: high HDL-C (H-HDL) (${\geq}$ 1.3 mmol/L, n=32) and low HDL-C (L-HDL) (< 1.3 mmol/L, n=57). Plasma lipids, insulin, adiponectin, apolipoproteins, oxidized LDL, Lipoprotein(a), and lipoprotein size and subfractions were measured, and 3-d dietary records were used to assess macronutrient intake. Women with L-HDL had higher sugar intake and glycemic load (P< 0.05), higher plasma insulin (P< 0.01), lower adiponectin (P< 0.05), and higher numbers of atherogenic lipoproteins such as large VLDL (P < 0.01) and small LDL (P<0.001) than the H-HDL group. Women with L-HDL also had larger VLDL and both smaller LDL and HDL particle diameters (P<0.001). HDL-C was positively correlated with LDL size (r=0.691, P<0.0001) and HDL size (r=0.606, P<0.001), and inversely correlated with VLDL size (r=-0.327, P<0.01). We concluded that L-HDL could be used as a marker for increased numbers of circulating atherogenic lipoproteins as well as increased insulin resistance in women who are already at risk for CVD.
The Journal of the Korea institute of electronic communication sciences
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v.8
no.5
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pp.779-784
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2013
This study aims to look into the relationship between the parents's family history of diabetes and factors of metabolic syndrome focusing on Korean adult males with a family history of diabetes. The data used for the study was collected from the 2010 Korea National Health and Nutrition Examination Survey. The subjects of the study totaled 2,045. For statistical analysis, double sampling general linear regression was used and the statistical significance was p<0.05. As a result of a multi-variate analysis with general characteristics corrected, the following was discovered: when fathers had a family history of diabetes, girth increased by 2.5cm, fasting blood sugar(glu) increased by 9.6mg/dL and neutral fat increased by 41.6mg/dL When the mothers had a family history of diabetes, girth increased by 2.4cm, fasting blood sugar(glu) increased by 15.4mg/dL, and the neutral fat increased by 27.2mg/dL. In conclusion, when the fathers had a family history of diabetes, their children's girth, fasting blood sugar and neutral fat were significantly higher, and when the mothers had a family history of diabetes, their children's girth and fasting blood sugar were significantly higher.
This study was conducted to investigate the prevalence of metabolic syndrome(MS) and characteristics of nutrient intake in MS subjects by gender and age. The subjects were 957(447 men and 510 women) who visited medical center for regular medical check-up. The diagnosis of MS subjects was adapted from NCEP-ATPIII with blood glucose, cholesterol, triglycerides, and blood pressure and Aisa-Pacific definition with waist-circumference. Anthropometric and biochemical measurements were practiced, then the nutrient intake analysis was assessed through the 24-hour recall method. The MS prevalence of all subjects was 10.3% in average -17% in men and 4.5% in women, respectively. The energy intake in MS group was 2,047.1 kcal and 1,699.5 kcal for normal group, showing significantly higher in MS compared to normal subjects. For intakes of animal fat, cholesterol, and sodium, MS group were significantly higher than normal group. In respect of gender, men subjects of MS group showed significantly higher nutrient intakes than normal group for energy, fat, and cholesterol. Women subjects of MS group showed higher intakes for energy, carbohydrate, and protein. For 30s, MS group showed higher intakes of energy, animal fat, and cholesterol than normal group. Fat and cholesterol for 40s and energy, carbohydrate, vegetable fat for 50s, MS group showed significantly higher intakes than normal group. In summary, MS group showed higher intakes of energy, animal fat, cholesterol, and sodium than normal group.
Objectives: This study evaluated the nutritional status and prevalence of metabolic syndrome of the people who participated in the KNHANES according to the number of household members. They were assessed by using information from the 2013~2014 Korea National Health and Nutrition Examination Survey (KNHANES). Methods: A total of 6,088 persons aged 19 years and over participated in 2013~2014 KNHANES, and they were classified into three groups according to the number of household members (single-person, two-person, three-person & over). The dietary behavior, nutritional status, health-related factors and the prevalence of metabolic syndrome of the subjects were investigated with using information from the survey questionnaires of KNHANES. The nutrient intake data of the subjects were obtained by the 24-hour recall method and this was analyzed for evaluating the nutrition adequacy ratio and the index of nutritional quality. The prevalence of metabolic syndrome among the subjects, and according to the study groups, was estimated using the blood and physical measurement data of the subjects. Results: As for EQ-5D index available for all the health states generated by the EQ-5D descriptive system, the single-person household member was the lowest among all the household types. The index of nutrition quality for protein, crude fiber, calcium, phosphorus, potassium, riboflavin and vitamin C in the single-person household was lower than that of the two-person or the three-person and over households (p<0.001). The mean adequacy ratio of single-person households was significantly decreased compared with that of the other types of households (p<0.001). The prevalence of metabolic syndrome was higher in the single-person households than that in the multiple-person households (p<0.001). Conclusions: These results showed that dietary behaviors, nutrition status and health status might be influenced by the number of household members. The results from this study would be useful for improving Korean people's dietary life and health status by implementing evidence-based, specialized intervention for the members of diverse types of households.
Hwang, Yu Im;Sung, Na Young;Koo, Hwa Seon;Cha, Sun Hwa;Park, Chan Woo;Kim, Jin Yeong;Yang, Kwang Moon;Song, In Ok;Koong, Mi Kyoung;Kang, Inn Soo;Kim, Hye Ok
Clinical and Experimental Reproductive Medicine
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v.40
no.3
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pp.135-140
/
2013
Objective: To evaluate correlations between serum anti-M${\ddot{u}}$llerian hormone (AMH) levels, phenotypes of polycystic ovary syndrome (PCOS), obesity, and metabolic parameters in patients with PCOS. Methods: A total of 175 patients with PCOS were diagnosed according to the Rotterdam Consensus were included. Exclusion criteria were age over 40, FSH>25 mIU/mL, and 17a-OHP>1.5 ng/mL. The Phenotypes of PCOS were divided into a severe form (oligo-anovulation, ANOV/hyperandrogenism/polycystic ovary morphology [PCOM]; n=59) and a mild form without HA (ANOV/PCOM, n=105). The serum AMH levels were classified into 3 groups (<5 vs. 5-10 vs. >10 ng/mL). Obesity was defined as body mass index (BMI) ${\geq}25kg/m^2$ (n=34). Results: The mean age was $25.9{\pm}5.7$ year and mean AMH level was $10.1{\pm}5.4$ ng/mL. The BMI ($kg/m^2$) was higher in group 1 ($24.2{\pm}6.3$) than in group 2 ($21.9{\pm}4.3$, p=0.046) or group 3 ($21.6{\pm}3.3$, p=0.019). There was no difference among the three groups in age, menstrual interval, antral follicle counts, androgens, or other metabolic parameters. The obesity group showed significantly lower AMH ($7.7{\pm}3.9$ ng/mL vs. $10.7{\pm}5.6$ ng/mL), p=0.004) and low-density lipoprotein levels ($93.1{\pm}21.2$ mg/dL vs. $107.5{\pm}39.3$ mg/dL, p=0.031), and showed higher total T ($0.74{\pm}0.59$ L vs. $0.47{\pm}0.36$ ng/mL, p=0.001), free T ($2.01{\pm}1.9$ vs. $1.04{\pm}0.8$ pg/mL, p=0.0001), and free androgen index ($6.2{\pm}7.9$ vs. $3.5{\pm}3.0$, p=0.003). After controlling for age factors and BMI, the serum AMH levles did not show any significant correlations with other hormonal or metabolic parmeters. Conclusion: For PCOS patients under the age 40, serum AMH is not negatively correlated with age. High serum AMH levels can not predict the phenotype of PCOS and metabolic disturbances in PCOS patients in the non-obese group. Further study might be needed to define the relation more clearly.
Young-Sun Kim;Woojeong Kim;Ji-Hoon Na;Young-Mock Lee
Clinical Nutrition Research
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v.12
no.3
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pp.169-176
/
2023
Glucose transporter type 1 (GLUT1) deficiency syndrome (DS) is a metabolic brain disorder caused by a deficiency resulting from SLC2A1 gene mutation and is characterized by abnormal brain metabolism and associated metabolic encephalopathy. Reduced glucose supply to the brain leads to brain damage, resulting in delayed neurodevelopment in infancy and symptoms such as eye abnormalities, microcephaly, ataxia, and rigidity. Treatment options for GLUT1 DS include ketogenic diet (KD), pharmacotherapy, and rehabilitation therapy. Of these, KD is an essential and the most important treatment method as it promotes brain neurodevelopment by generating ketone bodies to produce energy. This case is a focused study on intensive KD nutritional intervention for an infant diagnosed with GLUT1 DS at Gangnam Severance Hospital from May 2022 to January 2023. During the initial hospitalization, nutritional intervention was performed to address poor intake via the use of concentrated formula and an attempt was made to introduce complementary feeding. After the second hospitalization and diagnosis of GLUT1 DS, positive effects on the infant's growth and development, nutritional status, and seizure control were achieved with minimal side effects by implementing KD nutritional intervention and adjusting the type and dosage of anticonvulsant medications. In conclusion, for patients with GLUT1 DS, it is important to implement a KD with an appropriate ratio of ketogenic to nonketogenic components to supply adequate energy. Furthermore, individualized and intensive nutritional management is necessary to improve growth, development, and nutritional status.
Polycystic ovary syndrome (PCOS) is a common endocrine and metabolic disorder among reproductive-age women. As a leading cause of anovulatory infertility, it complicates fertility treatments, including in vitro fertilization. The widely accepted 2003 Rotterdam diagnostic criteria for PCOS include sub-phenotypes based on variations in androgen excess, ovulatory dysfunction, and polycystic ovarian morphology. In this systematic review, we examined the impacts of inositol and vitamin D on fertility in PCOS. Adhering to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses 2020 guidelines, we used relevant keywords to comprehensively search databases including PubMed, Google Scholar, and MDPI. From an initial pool of 345 articles, 10 met the inclusion criteria. The articles suggest that vitamin D and inositol, particularly myo-inositol and D-chiro-inositol, may represent therapeutic options for PCOS. Vitamin D influences ovarian follicular development, glucose regulation, and insulin sensitivity. When combined with metformin therapy, it is associated with improved menstrual regularity and ovulation. Inositol is crucial for cellular signaling, energy metabolism, glucose regulation, and fertility. This systematic review underscores the importance of investigating inositol and vitamin D within a PCOS management strategy, given the disorder's prevalence and impacts on fertility and metabolic health. Although these agents show promise, additional research could clarify their mechanisms of action and therapeutic benefits. This review emphasizes the need for exploration of effective treatments to improve the quality of life among individuals with PCOS. Inositol and vitamin D represent potential options, but more studies are required to elucidate their roles in the management of this condition.
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