Multiple enzyme defects in mitochondria of a case with congenital lactic acidosis and hyperammonaemia.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to T Tsuboi.
Explore the source record for details and available documents.
Five strains of Rickettsia tsutsugamushi were isolated in Ehime Prefecture during December 1987 to January 1990. Of these, two strains, the Yamazaki and Noma-3, were isolated at Noma area of Imabari city and three strains, the Kakiwara-10, -11, -12, at Kakiwara area of Uwajima city. The Yamazaki strain was isolated from a patient of tsutsugamushi disease and the other strains from wild rodents (Apodemus speciosus). These strains showed virulence in euthymic mice. The calculated LD50 of the Yamazaki and Kakiwara-10 strains showed 10(-3.0) and 10(-1.8), respectively. The immunofluorescent antibody test using thirty monoclonal antibodies to six representative strains, the Gilliam, Karp, Kato, Irie, Hirano and Shimokoshi, revealed that two strains isolated at Noma area, the Yamazaki and Noma-3, were identified as the Karp type and three strains at Kakiwara area, the Kakiwara-10, -11, -12, were identified as the Kato type. It was clarified that the serotypic differences were present among the strains isolated in Ehime Prefecture. Moreover, these five strains isolated in Ehime Prefecture did not react with the serotype-specific monoclonal antibodies to the Irie, Hirano and Shimokoshi strains known as the representative strains of so-called new type of tsutsugamushi disease, showing antigenic differences.
Bovine follicular oocytes collected from bovine ovaries were exposed to bovid herpesvirus 1 (BHV-1). After washings, these oocytes were cultured to mature. As a result BHV-1 could not be removed from the oocytes and could replicate in the oocytes with cumulus cells, but not in the oocytes without the cells. Moreover, the specific fluorescence for BHV-1 was detected in the cumulus cells by a indirect immunofluorescent technique. Therefore these findings suggested BHV-1 could be absorbed in the oocytes but the replication of BHV-1 was done in the cumulus cells.
No differences were observed in the isozyme patterns of 4 enzymes examined between fresh samples stored at -80 C and samples stored at room temperature for 10 days after lyophilization, which supports the validity of comparing lyophilized samples to fresh frozen tissue. Mature proglottids as well as plerocercoids of Spirometra erinacei from Japan and Australia were indistinguishable by comparison of isozyme patterns after isoelectric focusing. The isozyme patterns of acid phosphatase, glucosephosphate isomerase (GPI), and mannosephosphate isomerase from plerocercoids of Spirometra mansonoides were distinctly different from those of plerocercoids of S. erinacei. The adenylate kinase isozyme patterns of the mature proglottids of S. mansonoides were also distinctly different from those of the mature proglottids and the plerocercoids of S. erinacei. The GPI isozyme pattern of the mature proglottids of S. mansonoides was also distinguishable from the GPI patterns of those of S. erinacei. These electrophoretic data suggest that the S. erinacei from Japan and Australia are closely related, if not identical, but that S. mansonoides is genetically distinct from S. erinacei.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Liver glycogen content decreased in golden hamsters experimentally infected with plerocercoids of Spirometra erinacei. The activity of glycogen synthase a decreased significantly in infected animals, whereas that of glycogen phosphorylase a was not significantly affected. These observations suggest that changes in glycogen content were not attributable to increased glycogenolysis, but rather resulted from suppressed glycogenesis. Plasma immunoreactive insulin (IRI) concentrations in infected animals were slightly lower than those in controls, but the differences were not statistically significant. Plasma glucagon concentrations were significantly higher in infected animals. These results suggest that the suppression of glycogen synthase activity in S. erinacei-infected hamsters was attributable to enhanced levels of glucagon and that enhanced secretion of glucagon was caused by parasite-induced hypoglycemia.
Determining the clinical prognosis a 16-year follow-up study of a clinic-based FC cohort was made. The cohort comprises 528 FC children under 5 years of age at first clinic visit. Thirty-nine patients (7.4%) were found to have developing non-febrile seizures (FCC). Discrimination formula was applied; differences in actual cumulative FCC rates differed: a) whether the discriminant score was plus or minus (15%, 31/208 and 2.5% 8/320, respectively; p less than 0.001); b) whether the discriminant score was plus or minus in the group with no medication (47%, 22/47 and 3%, 6/229; p less than 0.001); and c) whether the treatment was applied or not in the group with plus value (6%, 9/161 and 47%, 22/47; p less than 0.001). No difference was detected whether the treatment was introduced or not in the group with a minus discriminant score (2%, 2/91 and 3%, 6/229, ns). The effective prediction and prevention for the FCC development were thus proved. Correlation between the number of predictive eight-risk factors and rates of FCC development are analyzed.
Explore the source record for details and available documents.
Children with febrile convulsions (FC) including 46 twin pairs, 1913 families including 393 sibling pairs, and 42 three-generation FC kindreds have been studied. Twin studies: (1) The pairwise concordance rate for FC was 69% (18/26 pairs) in monozygotic (MZ) and 20% (4/20 pairs) in dizygotic (DZ) twins (P less than 0.01). (2) The intra-pair similarity of clinical symptoms in 18 concordant MZ twin pairs showed a positive significant correlation, particularly in 4 items--duration of seizure, exogenous factors, intelligence level, and background EEG abnormality. These correlations were greater than those in sibling pairs. (3) No evident cause for discordance was detected in 8 discordant MZ twin pairs, and many dissimilar symptoms were observed in 4 concordant DZ twin pairs. Sibship studies: A large positive correlation of some clinical symptoms was observed in sibling pairs concordant for FC: age at onset of FC, degree of fever, duration of seizure, exogenous factors, and background EEG abnormality (r = +0.2- +0.6). Family history analysis: Morbidity risk among near relatives (17% in parents, 23% in siblings) than in second- (6.1%) or third-degree relatives (4.6%). The difference was found between: sibling greater than parents, uncles greater than aunts, male cousins greater than female cousins. Segregation analysis showed maternal preponderance. In 42 three-generation kindreds the morbidity risk was higher in siblings (32%), uncles/aunts (14%), and cousins (6.4%) than in relatives of other probands. Characteristic findings in FC patients with family history: Characteristic findings in FC patients with an FC parent or sibling, compared with those with no family history, were early onset of FC, lower degree of fever, longer duration of seizure, many recurrences, FC recurrence after age 3, and background EEG abnormality. Similar findings were more markedly observed in 42 3-generation kindreds. Mode of inheritance: A multifactorial mode of inheritance for FC receives some support from this study, and the heritability was estimated as 75%.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.