• Journal of mucopolysaccharidosis and rare diseases
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• v.4 no.1
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• pp.21-25
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• 2018

Fabry disease is a hereditary lysosomal storage disorder caused by the reduction or absence of lysosomal enzyme alpha-galactosidase A and the accumulation of glycosphingolipids, such as globotriaosylceramide (Gb3), in various organs, including the heart. The prevention of cardiac involvement in Fabry disease can only be achieved by enzyme replacement therapy (ERT), and the method of assessing the efficacy of ERT should be confirmed. Changes in the electrocardiogram, such as the shortening of PQ interval, prolongation of QTc and repolarization abnormalities as well as left ventricular hypertrophy in voltage criteria, can be used to identify Fabry disease patients; however, the usefulness of electrocardiograms for evaluating the efficacy of ERT is limited. The assessment of left ventricular hypertrophy using echocardiography has been established to evaluate the efficacy of ERT during long-term period. A new technique involving speckled tracking method might be useful for detecting early cardiac dysfunction and identifying the effect of ERT for a relatively short period. The estimation of left ventricular hypertrophy using cardiac magnetic resonance (CMR) is also useful for assessing the efficacy of ERT. Identifying late gadolinium enhancement in CMR may affect the effectiveness of ERT, and the new technique of T1 mapping might be useful for monitoring the accumulation of Gb3 during ERT. Histopathology in cardiac biopsy specimens is another potentially useful method for identifying the accumulation of GB3; however, the use of histopathology to evaluate of the efficacy of ERT is limited because of the invasive nature of an endomyocardial biopsy.

• Journal of Animal Science and Technology
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• v.66 no.2
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• pp.438-441
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• 2024

The Enterococcus faecium (E. faecium) strain AK_C_05 was isolated from cheonggukjang, the Korean traditional food, collected from a local market in South Korea. In this report, we presented the complete genome sequence of E. faecium strain AK_C_05. The genome of E. faecium strain AK_C_05 genome consisted of one circular chromosome (2,691,319 bp) with a guanine + cytosine (GC) content of 38.3% and one circular plasmid (177,732 bp) with a GC content of 35.48%. The Annotation results revealed 2,827 protein-coding sequences (CDSs), 18 rRNAs, and 68 tRNA genes. It possesses genes, which encodes enzymes such as alpha-galactosidase (EC 3.2.1.22), beta-glucosidase (EC 3.2.1.21) and alpha-L-arabinofuranosidase (EC 3.2.1.55) enabling efficient utilization of carbohydrates. Based on Clusters of Orthologous Groups analysis, E. faecium strain AK_C_05 showed specialization in carbohydrate transport and metabolism indicating the ability to generate energy using a variety of carbohydrates.

• Journal of Microbiology and Biotechnology
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• v.16 no.10
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• pp.1613-1621
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• 2006

Isolation and identification of algal lytic bacteria were carried out. Nine strains of algal lytic bacteria were isolated by the double-layer method using Anabaena flos-aquae as a sole nutrient. The isolate, AFK-13, showing the highest algal lytic activity was identified as Sinorhizobium kostiense based on the l6S rDNA sequence. The algal lytic experiments of the culture supernatants of AFK-13 demonstrated that the bacterial cell growth reached a maximum at 36-h culture, but the supernatant of 72-h culture exhibited the highest activity. Components among the extracellular products in the crude enzyme of the supernatant from S. kostiense AFK-13 culture were responsible for degradation of cell walls of Anabaena flos-aquae. Algal lytic assay tests of the culture supernatants suggest that the main substances for algal lytic activity could be proteinaceous. The activity of glucosidase was observed highly by polysaccharolytic analysis using the crude enzyme from S. kostiense AFK-13, whereas activities of galactosidase, mannosidase, rhamnosidase, and arabinosidase were also detected in low levels. The molecular weights (MW) of ${\alpha}-\;and\;{\beta}$-glucosidases were estimated to be approximately 50-100 kDa by the ultrafiltration method.

• Proceedings of the KSAR Conference
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• 2001.03a
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• pp.43-43
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• 2001

This study evaluated the effect of fertilization-promoting peptide (FPP) on fertilizing ability and glycosidase activity in vitro of spermatozoa frozen-thawed in pig, Using chlortetracycline fluorescence analysis, the various glycosidase analyses and the oocyte penetration test, we have obtained evidence that FPP can promote the fertilizing ability and glycosidase activity of frozen-thawed spermatozoa in vitro. When frozen-thawed spermatozoa was washed with different concentrations of FPP, there were significantly (P<0.05) more acrosome-reacted in medium with 100 nM than 0, 50, 200 and 400 nM. The penetration rates were also highest in medium containing with 100 nM FPP (P<0.05). On the other hand, the $\beta$-N-acetylglucosaminidase activity was at least twofold higher than other glycosidase. In same glycosidase, however, there were no difference in medium with different concentrations of FPP In another experiment, spermatozoa preincubated in medium with or without FPP for 0, 1, 2, 3 and 4 h were inseminated with oocytes matured in vitro. The percentages of spermatozoa that reached acrosome reaction were affected by preincubation and were higher in medium with that than without FPP. When oocytes were inseminated with spermatozoa preincubated in medium with and without FPP during the different periods, however, penetration rates were decreased with preincubation periods of spermatozoa. On the other hand, when the sperm-oocyte were cultured for 4, 8, 12, 16, 20 and 24 h, the penetration rates were higher in spermatozoa preincubated with that than without FPP and had a tendency to increase as time of culture periods. However, The activities of $\alpha$-fucosidase, $\alpha$ -mannosidase, $\beta$-galactosidase and N-acetyl- $\beta$-D-glucosaminidase were higher in medium with that than without FPP regardless of periods of sperm preincubation and sperm-oocyte culture. These results suggest that FPP may play a positive role in promoting of sperm function and glycosidase activity in vitro in pig.

• Asian-Australasian Journal of Animal Sciences
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• v.21 no.7
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• pp.1053-1058
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• 2008

A total of 392 twenty eight week-old laying hens was used to study the effects of dietary inclusion of solvent-extracted palm kernel cake (PKC) (0%, 12.5% and 25%) and enzyme (mixture of mannanase, ${\alpha}$-galactosidase and protease) supplementation (0 kg/t, 1 kg/t and 2 kg/t) on the performance of laying hens. The levels of PKC did not significantly influence nitrogen corrected true metabolizable energy (TMEn) of the diets. Enzyme-supplemented PKC had significantly higher AME and TMEn values than PKC diets with no enzyme supplementation. Dietary inclusion of 12.5% and 25% PKC in the diets of laying hens did not adversely affect mean egg production or daily egg mass. However, layers consumed significantly more PKC-based diets and had significantly poorer feed conversion ratios (FCR) than controls. However, the feed intake and FCR of hens provided the 12.5% PKC-based diets with enzyme supplementation at 1 kg/t did not differ from the controls. Dietary inclusion of PKC or enzyme did not affect eggshell quality, but egg yolk colour was significantly paler when layers were fed the 25% PKC diet.

• Preventive Nutrition and Food Science
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• v.14 no.3
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• pp.252-259
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• 2009

We previously isolated Bacillus strains with high fibrinolytic activities (FAs) from cheonggukjang prepared by traditional ways. To test their potential as starters for cheonggukjang, soybean was fermented for 72 hr at $37^{\circ}C$ with each isolate and a control lab strain: B. subtilis CH3-25 (BS3-25), B. amyloliquefaciens CH51 (BA51), B. amyloliquefaciens CH86-1 (BA86-1), and B. subtilis 168 (BS168, control, lab strain). Viable cell numbers of all cheonggukjang samples rapidly increased and reached about $10^9$ CFU/g after 6 hr. During 72 hr, the initial pH of 6.3 rapidly increased to 8.1$\sim$8.2 for cheonggukjang fermented with BS3-25 or BA86-1, and 7.3 for those with BA51 or BS168. FAs and protease activities (acid, neutral, and alkaline) rapidly increased in cheonggukjang fermented with BS3-25, BA51, or BA86-1 during the first 12 hr. On the other hand, those of cheonggukjang fermented with BS168 slightly increased during the first 36 hr. There were significant changes in acid and neutral protease activities in cheonggukjang fermented with BA51 or BA86-1 during the 24 hr. Rapid increases of $\beta$-glucosidase activity corresponded well with rapid increases of $\alpha$-amylase and $\alpha$-galactosidase activities in addition to increases in antioxidant activities and the TPCs (total phenolic contents). The highest increase in the TPCs was observed in cheonggukjang fermented with BA86-1 while the least was that fermented with BS168.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.14 no.1
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• pp.37-41
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• 2014

Fabry disease is an X-linked disease caused by deficiency of the lysosomal enzyme alpha-galactosidase A. Affected males present anhydrosis, acroparesthesia and angiokeratoma, and subsequently cardiac, cerebral and renal complications are followed. Females and atypical variants show heterogeneous clinical symptoms. In 2001, two recombinant enzymes were approved for Fabry disease: agalsidase alpha and agalsidase beta. Since the introduction of enzyme replacement therapy (ERT), the number of long-term follow-up studies has been reported. Long-term ERT showed effectiveness on renal function in patients with chronic kidney disease, decrease or stabilization of left ventricular mass, and improvement of pain and quality of life. However, there were limited effects on cerebrovascular events and their mortality. Current literatures on the clinical effect of ERT have reported limited datain adult patients who have already advanced disease. Therefore, further study for pre-symptomatic patients and atypical variants is needed to verify the impact of ERT. This review summarized recent progresses in ERT and limitations of long-term effect of ERT in patients with Fabry disease.

• Journal of the Korean Applied Science and Technology
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• v.33 no.2
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• pp.286-292
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• 2016

1, 2-Hexanediol galactoside (HD-gal) has been synthesized from 1, 2-hexanediol (HD), a cosmetic preservative, using recombinant Escherichia coli ${\beta}$-galactosidase (${\beta}$-gal) at the high lactose concentration (300 g/l). To confirm the molecular structure of synthesized HD-gal, NMR ($^1H$- and $^{13}C$-) spectroscopy and mass spectrometry of HD-gal were conducted. $^1H$ NMR spectrum of HD-gal showed multiple peaks corresponding to the galactocyl group, which is an evidence of galactocylation on HD. Downfield proton peaks at ${\delta}_H$ 4.44 ppm and multiple peaks from ${\delta}_H$3.96~3.58 ppm were indicative of galactocylation on HD. Up field proton peaks at ${\delta}_H$ 1.60~1.35 ppm and 0.92 ppm showed the presence of $CH_2$ and $CH_3$ protons of HD. $^{13}C$ NMR spectrum revealed the presence of 21 carbons suggestive of ${\alpha}$- and ${\beta}$-anomers of HD-gal. Among 12 carbon peaks from each anomers, the 3 peaks at dC 68.6, 60.9 and 13.2 ppm were assigned to be overlapped showing only 21 peaks out of total 24 peaks. The mass value (protonated HD-gal, m/z = 281.1601) from mass spectrometry analysis of HD-gal, and $^1H$ and $^{13}C$ NMR spectral data were in well agreement with the expecting structure of HD-gal. For further study, the minimum inhibitory concentrations (MICs) of HD-gal against bacteria will be investigated, and, in addition, cytotoxicity to human skin cells of HD-gal will be examined. It is expected that it will eventually be able to develop a new cosmetic preservative, which have low cytotoxicity against human skin cell and maintains antimicrobial effect.

• YAKHAK HOEJI
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• v.51 no.2
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• pp.96-102
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• 2007

Measurement of globotriaosylceramide (Gb3, ceramide trihexoside) in urine has clinical importance for monitoring after enzyme replacement therapy in Fabry disease patients. The disease is an X-linked lipid storage disorder that results from a deficiency of the enzyme ${\alpha}$-galactosidase A (${\alpha}$-Gal A). The lack of ${\alpha}$-Gal A causes an intracellular accumulation of glycosphingolipids, mainly Gb3. A simple, rapid, and highly sensitive analytical method for Gb3 in urine was developed without labor-extensive pre-treatment by electrospray ionization MS/MS (ESI-MS/MS). Only simple 5-fold dilution of urine is necessary for the extraction and isolation of Gb3 in urine. Gb3 in diluted urine was dissolved in dioxane containing C17:0 Gb3 as an internal standard. After centrifugation it was directly injected and analyzed through guard column by in combination with multiple reaction monitoring mode of ESI-MS/MS. Eight isoforms of Gb3 were completely resolved from urine matrix. C24:0 Gb3 occupied 50% of total Gb3 as a major component in urine. Linear relationship for Gb3 isoforms was found in the range of 0.005${\sim}$5.0 ${\mu}$g/ml. The limit of detection (S/N=5) was 0.005 ${\mu}$g/ml and limit of quantification was 0.05 ${\mu}$g/ml for C24:0 Gb3 with acceptable precision and accuracy. Correlation coefficient of calibration curves for 8 Gb3 isoforms ranged from 0.9598 to 0.9975. This method could be useful for rapid and sensitive 1st line Fabry disease screening, monitoring and/or diagnostic tool for Fabry disease.

• Journal of Embryo Transfer
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• v.32 no.3
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• pp.209-220
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• 2017

The estrogen-mediated effect of mesenchymal stem cells (MSCs) is a highly critical factor for the clinical application of MSCs. However, the present study is conducted on MSCs derived from adult donors, which have different physiological status with steroid hormonal changes. Therefore, we explores the important role of $17{\beta}$-estradiol (E2) in MSCs derived from female and male newborn piglets (NF- and NM-pBMSCs), which are non-sexually matured donors with steroid hormones. The results revealed that in vitro treatment of MSCs with E2 improved cell proliferation, but the rates varied according to the gender of the newborn donors. Following in vitro treatment of newborn MSCs with E2, mRNA levels of Oct3/4 and Sox2 increased in both genders of MSCs and they may be correlated with both estrogen receptor ${\alpha}$ ($ER{\alpha}$) and $ER{\beta}$ in NF-pBMSCs, but NM-pBMSCs were only correlated with $ER{\alpha}$. Moreover, E2-treated NF-pBMSCs decreased in ${\beta}$-galactosidase activity but no influence on NM-pBMSCs. In E2-mediated differentiation capacity, E2 induced an increase in the osteogenic and chondrogenic abilities of both pBMSCs, but adipogenic ability may increased only in NF-pBMSCs. These results demonstrate that E2 could affect both genders of newborn donor-derived MSCs, but the regulatory role of E2 varies depending on gender-dependent characteristics even though the original newborn donors had not been affected by functional steroid hormones.