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Biomedical subjects

Y S Shin

Publications and source records attributed to Y S Shin.

At least 55 records · Page 3Linked to original sources
PubMed1998

Mutations in the liver glycogen phosphorylase gene (PYGL) underlying glycogenosis type VI.

Deficiency of glycogen phosphorylase in the liver gives rise to glycogen-storage disease type VI (Hers disease; MIM 232700). We report the identification of the first mutations in PYGL, the gene encoding the liver isoform of glycogen phosphorylase, in three patients with Hers disease. These are two splice-site mutations and two missense mutations. A mutation of the 5' splice-site consensus of intron 14 causes the retention of intron 14 and the utilization of two illegitimate 5' splice sites, whereas a mutation of the 3' splice-site consensus of intron 4 causes the skipping of exon 5. Two missense mutations, N338S and N376K, both cause nonconservative replacements of amino acids that are absolutely conserved even in yeast and bacterial phosphorylases. We also report corrections of the PYGL coding sequence, sequence polymorphisms, and a partial PYGL gene structure with introns in the same positions as in PYGM, the gene of the muscle isoform of phosphorylase. Our findings demonstrate that PYGL mutations cause Hers disease, and they may improve laboratory diagnosis of deficiencies of the liver phosphorylase system.

Amino Acid Sequence↗
PubMed1998

The infusion rate of mivacurium and its spontaneous neuromuscular recovery in magnesium-treated parturients.

UNLABELLED: Magnesium (Mg) enhances the activity of nondepolarizing neuromuscular blocking drugs. However, no interaction between mivacurium and magnesium has been described. Therefore, we sought to determine the effect of the influence of Mg on the infusion rate of mivacurium and its spontaneous recovery. We studied 24 parturients who had undergone cesarean section under general anesthesia. Those who had been given MgSO4 for the treatment of preeclampsia were assigned to the Mg group (n = 12), and the other normal parturients were assigned to the NonMg group (n = 12). In both groups, the train-of-four (TOF) response to stimuli of the ulnar nerve was measured at intervals of 15 s. Anesthesia was induced with thiopental and succinylcholine. In both groups, a bolus dose of mivacurium 0.06 mg/kg was administered when the first twitch of TOF (T1) reached 100% after the succinylcholine injection. When the electromyographic response after mivacurium had recovered to approximately 5%-10% of the baseline, a continuous infusion of mivacurium was given to maintain 93%-97% neuromuscular blockade. The plasma concentration of Mg in blood of the Mg group was 4.0 1.0 mEq/L, higher than that (1.4 mEq/L) of the NonMg group (P < 0.01). The infusion rates of mivacurium of Mg and NonMg groups were 1.6 and 5.4 mEq x kg(-1) x min(-1), respectively. In addition, the recovery indexes of the Mg and NonMg groups were 12.9 and 4.3 min, respectively. We conclude that a smaller dose of mivacurium should be infused to patients receiving Mg. IMPLICATIONS: Magnesium, used as a standard therapy for severe toxemia, may act to enhance muscle relaxants such as mivacurium, a short-acting drug used in general anesthesia. Among women undergoing a cesarean section who were given a magnesium pretreatment, the infusion rate of mivacurium required to obtain relaxation was lower than that among women who did not receive pretreatment.

Cholinesterases↗
PubMed1998

Prevalence estimation of dementia in a rural area of Korea.

OBJECTIVES: A cross-sectional survey was conducted to estimate prevalence of dementia and to determine factors related to dementia among residents in a rural area, Yonchon county, Korea, in 1993. PARTICIPANTS: Of the study population more than 65 years of age (n = 2171), 1674 persons participated in the cross-sectional survey using the Korean version of the Mini-Mental State Examination (MMSE-K). DESIGN: Subjects were defined as having dementia according to NINCDS-ADRDA criteria through direct interview by psychiatrists. A total of 436 subjects were selected randomly according to MMSE-K scores, and cases were confirmed as having either Alzheimer's or vascular dementia. RESULTS: The adjusted prevalence of dementia was estimated at 9.5%. The prevalence of total dementia was 8.8% for men and 9.9% for women. The prevalence of Alzheimer's disease was 3.2% for men and 5.3% for women and that of vascular dementia 3.1% and 2.1%, respectively. Age-standardized prevalence of Alzheimer's disease was 4.5%, whereas that of vascular dementia was 2.5%. Using DSM-III-R criteria to stratify dementia patients according to severity showed the prevalence of mild, moderate, and severe Alzheimer's disease was 3.4%, 0.7%, and 0.5%, respectively. CONCLUSION: These results showed that the prevalence of dementia in Korea was similar to its presence in Western countries but different from that previously reported in other Asian countries, e.g., China and Japan.

Aged↗
PubMed1998

Acute effects of chundosunbup qi-training on blood concentrations of TSH, calcitonin, PTH and thyroid hormones in elderly subjects.

The present study investigated how the systemic treatment of a programmed exercise, ChunDoSunBup (CDSB) Qi-training, affects the secretion of thyroid and parathyroid hormones in elderly subjects (10 male and 5 female). Plasma concentrations of thyroid stimulating hormone (TSH), triiodothyronine (T3), thyroxine (T4), parathyroid hormone (PTH), ionized calcium, and calcitonin were determined. CDSB Qi-training induces a slight increase in TSH. Both T4 and T3 were increased at the mid-time of CDSB Qi-training (p < 0.05). There were significant correlations only between T3 and T4 at mid-training. This shows that increase in the plasma level of T3 was associated with the secretion of T4. The plasma concentrations of calcitonin and PTH were increased at mid-time and post-time of CDSB Qi-training. But ionized calcium was decreased slightly by CDSB Qi-training. These results suggest that Qi-training modulates the secretion of thyroid hormones, calcium metabolism, and parathyroid hormones in the elderly. However, whether the long-term practice of CDSB Qi-training might change bone metabolism and have longitudinal effects on the thyroid hormone of the elderly need further investigation.

Aged↗
PubMed1998

Urinary soluble HLA class I antigen in patients with minimal change disease: a predictor of steroid response.

In primary minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS), increased lymphocyte reactivity to renal antigens has been defined. Soluble HLA class I antigen (sHLA-I) is actively secreted by T and B lymphocytes when they are stimulated by mitogens, antigens and lymphokines. To determine if serum and urine sHLA-I levels could predict steroid response in patients with MCD and differentiate those from FSGS, we have investigated 45 healthy controls, biopsy-proven 17 patients with MCD (edema and 24-hour urine protein > 3.5 g/day), 8 patients with FSGS (24-hour urine protein > 1 g/day) and 10 patients with membranous nephropathy (MGN) (24-hour urine protein > 1 g/day). Before and after prednisone therapy (1 mg/kg/day or 2 mg/kg/EOD for 8 weeks), the levels of serum and urinary sHLA-I were measured by ELISA (sHLA-STAT; Sangstat Co., Calif., USA). After 8 weeks of treatment, 10 patients with MCD were responders (MCD-CR) while the other 7 patients with MCD were nonresponders (MCD-NR). Three of 7 patients with MCD-NR were re-biopsied and finally diagnosed as FSGS. They were included in the data of patients with FSGS. In healthy controls, serum sHLA-I was detected (415 +/- 256 ng/ml), but urinary sHLA-I was not. At entry, there were no differences in age, sex, serum Cr and 24-hour urine protein among the patients with MCD-CR, MCD-NR and FSGS, but serum albumin was significantly elevated in patients with FSGS and MGN (p < 0.05). Serum sHLA-I levels were notably elevated in MCD-CR (1,040 +/- 1,066 ng/ ml), in MCD-NR (668 +/- 315 ng/ml) and in FSGS (713 +/- 790 ng/ml), but not in patients with MGN (444 +/- 86 ng/ml) when compared with controls (p < 0.05). On the other hand, urinary sHLA-I levels in MCD-NR (541 +/- 239 ng/mg Cr) and in FSGS (457 +/- 239 ng/mg Cr) were significantly higher than those in MGN (125 +/- 28 ng/mg Cr) and in MCD-CR(100 +/- 42 ng/mg Cr, p < 0.05) and these substantial differences were maintained for 8 weeks. In all patients, serum and urinary sHLA-I levels were not reduced during 8 weeks of steroid therapy. We conclude that elevated serum and urinary sHLA-I levels reflect increased cellular immune response and disease activity in patients with MCD and FSGS. In patients with MCD, urinary sHLA-I may be an easily measurable indicator of predicting steroid response, while MCD-NR with high urinary sHLA-I levels might be re-evaluated for the possibility of FSGS.

Adolescent↗
PubMed1998

The nucleotide and predicted amino acid sequence of the fusion protein of recent isolates of canine distemper virus in Japan.

Analysis of the molecular properties of fusion (F) proteins of field isolates of canine distemper virus (CDV) by immunoprecipitation analysis revealed an identical molecular mass of F protein of 3 field isolates as well as the Onderstepoort laboratory strain. Sequencing showed that the F gene of a field isolate (the Yanaka strain) shared 90.1% and 95.7% identities with the Onderstepoort strain at nucleotide and amino acid levels, respectively. All of the 13 cysteine residues and 4 potential asparagine-linked glycosylation sites were completely conserved amongst these strains. These results indicate that the F proteins is much less heterogeneous than that observed in the hemagglutinin proteins of CDV.

Amino Acid Sequence↗
PubMed1998

Inhibition of calbindin D28K expression by cyclosporin A in rat kidney: the possible pathogenesis of cyclosporin A-induced hypercalciuria.

A recent study by Steiner et al. (Biochem Pharmacol 51: 253-258, 1996) demonstrated a decreased calbindin D28K expression in the kidneys of cyclosporin A (CsA)-treated rats. To evaluate the association of renal calcium handling with calbindin D28K expression in CsA-treated rats, two separate experiments (vehicle [VH] versus CsA groups, 1,25-dihydroxyvitamin D3 [VitD] versus VitD + CsA groups) were done simultaneously. CsA (25 mg/kg per d, subcutaneously) and VitD (0.5 microg/kg per d, subcutaneously) were given for 7 d. The CsA group showed decreased serum calcium, increased urine calcium excretion, and decreased calbindin D28K protein level and immunoreactivity compared with the VH group. The VitD + CsA treatment decreased serum calcium, increased urine calcium excretion, and decreased calbindin D28K protein level and immunoreactivity compared with the VitD alone. CsA treatment did not affect the serum parathyroid hormone and VitD levels. This study demonstrates an association of calbindin D28K expression with the urinary calcium excretion in CsA-treated rats, and suggests that decreased calbindin D28K expression may play a role in renal calcium wasting.

Animals↗
PubMed1997

Altered follicle stimulating hormone isoforms in female galactosaemia patients.

UNLABELLED: Many women affected with galactosaemia suffer from ovarian dysfunction and have elevated serum levels of follicle stimulating hormone (FSH). We have analysed FSH-glycoprotein isoforms from four galactosaemic and five healthy women. Besides the commonly found FSH species with a median isoelectric point (pI) of 4-5, the sera of the female galactosaemic patients contained qualitatively abnormal FSH isoforms with a pI close to neutral (6.4-7.0). The generally reduced galactosylation in patient samples was confirmed because sera of galactosaemic patients could incorporate 1.7 times more UDP-(14C)galactose than did healthy subjects. CONCLUSION: Our data indicate that the terminal disaccharides of FSH (a glycoprotein), galactose and sialic acid were partially deficient in three galactosaemic female patients with no galactose-1-phosphate uridyl transferase (GALT) activity in red cells. However, from a female patient with a residual GALT activity (a mild form of galactosaemia), no distinctive deficiency was observed. This again suggest an importance of GALT in retaining a correct FSH structure. Therefore the abundance of neutral FSH isoforms, which was described to have a higher binding affinity to its receptor and no capacity to activate cyclic adenosine mono-phosphate (cAMP), may cause a hormonal dysfunction in classical galactosaemia.

Adolescent↗
PubMed1997

Autosomal glycogenosis of liver and muscle due to phosphorylase kinase deficiency is caused by mutations in the phosphorylase kinase beta subunit (PHKB).

Glycogen storage disease due to phosphorylase kinase deficiency occurs in several variants that differ in mode of inheritance and tissue-specificity. This heterogeneity is suspected to be largely due to mutations affecting different subunits and isoforms of phosphorylase kinase. The gene of the ubiquitously expressed beta subunit, PHKB, was a candidate for involvement in autosomally transmitted phosphorylase kinase deficiency of liver and muscle. To identify such mutations, the complete PHKB coding sequence was amplified by RT-PCR of RNA isolated from blood samples of patients and analyzed by direct sequencing of PCR products. The characterization of mutations was complemented by PCR of genomic DNA. In one female and four male patients, we identified five independent nonsense mutations (Y418ter; R428ter; Y974H+E975ter; Q656ter in two cases), one single-base insertion in codon N421, one splice-site mutation affecting exon 31, and a large deletion involving the loss of exon 8. Although these severe translation-disrupting mutations occur in constitutively expressed sequences of the only known beta subunit gene of phosphorylase kinase, PHKB, they are associated with a surprisingly mild clinical phenotype, affecting virtually only the liver, and relatively high residual enzyme activity of approximately 10%.

Adolescent↗
PubMed1997

Molecular and phylogenetic analyses of the haemagglutinin (H) proteins of field isolates of canine distemper virus from naturally infected dogs.

We isolated three strains of canine distemper virus (CDV)--the Ueno, Hamamatsu, and Yanaka strains--from dogs in Japan and analysed the molecular properties of their haemagglutinin (H) proteins. Immunoprecipitation of all three strains with a monoclonal antibody revealed H proteins with molecular masses of 84 kDa, which differs from the molecular mass (78 kDa) of the H protein of the Onderstepoort vaccine strain. However, after tunicamycin treatment immunoprecipitation identified H proteins of identical molecular mass (68 kDa) for all three field isolates and the vaccine strain. Sequence analysis showed nine potential sites for asparagine-linked glycosylation in the H proteins of the new isolates, in contrast to four in the H protein of the Onderstepoort strain. Thus, variation in glycosylation of the H proteins of the isolates and the vaccine strain may cause differences in antigenicity of the viruses. Sequences of the H genes showed that the new Japanese isolates have 99% identity with each other, 95% with other European and American isolates (from seals, a German dog, a ferret and large felids) and 90% with the vaccine strain. Phylogenetically, the new Japanese isolates form one cluster which is separate from recent European or American isolates, all of which are distinct from vaccine strains.

Amino Acid Sequence↗
PubMed1997

Expression of the nucleocapsid protein gene of the canine distemper virus.

We constructed a cDNA clone of canine distemper virus (CDV) encoding an entire nucleoprotein (NP) gene, by means of the reverse transcription-polymerase chain reaction (RT-PCR). The cloned NP gene was inserted into the eucaryotic expression vector, pRVSV. After transfection of the plasmid into Vero cells, we examined the expression of CDV-specific NP antigen by means of indirect immunofluorescence assay (IFA) and Western blotting, using various antibodies against NP of CDV and an antiserum against NP of measles virus. The CDV-NP specific antigen was detected in the nuclei of the cells transfected with pRV-ON, by means of IFA with antibodies specific to the NP.

Animals↗
PubMed1997

Detection of proviruses and viral RNA in the early stages of feline immunodeficiency virus infection in cats: a possible model of the early stage of HIV infection.

Feline immunodeficiency virus (FIV) infection in cats has been reported to be a useful animal model for human AIDS studies, especially in the early stages of infection. We examined the temporal changes in provirus detection in peripheral blood mononuclear cells (PBMC) and the distribution of FIV-DNA and RNA in feline tissues by the polymerase chain reaction at 10, 35, 70 days after intravenous inoculation of FIV. Viral DNA in the PBMC was detected three to four weeks after infection and its fluctuation was demonstrated for the first time. Ten days after infection, before seroconversion, proviruses were detected only in the mesenteric lymph nodes and intestines. At 35 and 70 days after infection, after seroconversion, proviruses were detected in most lymphoid organs and the salivary glands, but the expression of FIV-RNA was limited to the thymus at 70 days after infection. These results show that FIV-RNA is transcribed from proviral DNA exclusively in the thymus at this stage. We suggest that the quantitative changes in detectable proviruses in the PBMC depend on the relation between the decrease in infected cells caused by cytolytic T lymphocytes and/or apoptosis and their increase caused by the release of a new supply of lymphocytes from the thymus.

Animals↗
PubMed1997

Management of poor-grade patients with ruptured intracranial aneurysm.

To formulate treatment strategies for poor-grade patients after aneurysmal subarachnoid hemorrhage (SAH), medical records were analyzed for 166 patients who were in Hunt and Hess Grade IV or V among 588 consecutive cases with ruptured intracranial aneurysm admitted during the past 5 years. Causes of unfavorable outcome (poor or dead) in those 166 patients were evaluated to improve the management outcome. Overall management results of the 166 poor-grade patients were favorable (good or fair) in 71 (42.8%), unfavorable in 95 (78 dead, 17 poor). Direct clipping was performed in 90 patients, and the results were favorable in 69 (76.7%) and unfavorable in 21 (23.3%). Surgery was not done in 76 patients because 41 were moribund on arrival, 15 deterioration due to rebleeding, 7 severe brain swelling, 5 serious medical illness, one severe delayed ischemic deficit (DID), and one cerebral infarction following angiography, and 6 refused surgery. Seven patients survived in non-surgery group (2 fair, 5 poor). Direct effects of aneurysm rupture (34.8%) and early rebleeding (34.8%) were the major causes of unfavorable outcome in Grade IV patients, while it was direct effect of aneurysm rupture (91.8%) in Grade V patients. It is suggested that as rebleeding is the only preventable cause of unfavorable outcome, urgent management is necessary to prevent rebleeding, especially for Grade IV patients. Grade IV patients should be treated aggressively with direct clipping for non-complex aneurysms or for patients with hematoma, and coil embolization for complex aneurysms without hematoma.

Aneurysm, Ruptured↗
PubMed1997

Risk factors for the development of NIDDM in Yonchon County, Korea.

OBJECTIVE: To determine the risk factors for the development of NIDDM in Yonchon County of Korea. RESEARCH DESIGN AND METHODS: We studied 1,193 Korean nondiabetic subjects at baseline who participated in a 2-year follow-up study on diabetes in Yonchon County. A 75-g oral glucose tolerance test was performed 2 years after the baseline examination. Age, sex, and anthropometric and metabolic characteristics at baseline were analyzed simultaneously as potential predictors of conversion to NIDDM. We also designed a nested case-control study to determine the role of hyperinsulinemia and/or hyperproinsulinemia in the conversion to NIDDM in patients with newly developed diabetes and control subjects matched for age, sex, BMI, and waist-to-hip-ratio. RESULTS: At 2 years, 67 subjects developed diabetes, as defined by World Health Organization criteria. The age-adjusted incidence was significantly higher in men (6.4%) than in women (3.0%), and the incidence increased as age increased in both sexes. Multiple logistic regression analysis revealed age, male sex, and fasting and 2-h glucose levels to be significant risk factors for the development of NIDDM, whereas waist-to-hip ratio and BMI were not. In a nested case-control study, baseline proinsulin but not insulin levels were significantly higher in subjects who progressed to NIDDM than in those who did not. CONCLUSIONS: In the Korean population, beta-cell dysfunction, as measured by high proinsulin levels, seems to be associated with subsequent development of NIDDM, whereas regional and general obesity and fasting insulin levels, which may be a surrogate for insulin resistance, were not.

Adult↗
PubMed1997

Morphological characteristics of the developing human brain during the embryonic period.

Many features of the developing nervous system are visible from external observations of intact human embryos. In this study, a photographic atlas from the 4th to the 7th week after ovulation (Carnegie stages 10-18) is provided. The neural folds began to fuse at stage 10, and the rostral and caudal neuropore were closed during stages 11 and 12, respectively. The three primary divisions of the brain were distinguishable before closing of the neural tube. The five secondary brain vesicles were formed during stages 14-15. The development of the cerebellum and cerebrum were first observed at stages 14 and 15, respectively. The mesencephalic flexure was seen at stage 12, and the cervical flexure and pontine flexure at stage 14. After stages 18-19, it became increasingly difficult to identify detailed features of the brain from the surface. Results from this study will help to correlate the characteristic findings of the developing central nervous system of human embryos from stereomicroscopical and light microscopical observations and to locate the exact parts of the developing human brain for other purposes.

Brain↗
PubMed1996

Pathogenicity and vaccine efficacy of a thymidine kinase-deficient mutant of feline herpesvirus type 1 in cats.

We constructed a recombinant feline herpesvirus type 1 (FHV-1) which was deleted in a defined region (450 bp) within the thymidine kinase (TK) gene (C7301dlTK) [Yokoyama et al. (1995) J Vet Med Sci 57: 709-714]. In this report, we carried out two experiments to assess the pathogenicity and vaccine efficacy of the recombinant C7301dlTK in cats. The first experiment showed that, following multiple inoculation of the recombinant C7301dlTK by intraocular, intranasal and oral routes, the virus was sufficiently attenuated in cats, although a high titer of the virus was recovered from target organs (eye, nose, and mouth). In the second experiment, two intramuscular vaccinations with the recombinant C7301dlTK protected cats to a significant degree against subsequent challenge with the parent FHV-1 strain C7301 at 4 weeks after the last vaccination. These results demonstrate that the recombinant C7301dlTK is effective as a live attenuated vaccine with a clear genetic marker.

Alphaherpesvirinae↗