[Roussy-Lévy syndrome showing onion bulb formation: report of three cases in a family].
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Biomedical subjects
Publications and source records attributed to S Kuroda.
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Streptomyces sp. No. 22 strain, which was freshly isolated from a soil sample froms Nossap Cape and considered to be closely related to Streptomyces alboviridis showed strong antitumor activity against L1210 culture established cells when incubated in glucose-starch bouillon under the usual conditions. An anti-L1210 substance was found at an early stage of mycelium development and in the filtrate. When extracted with organic solvents from the whole broth and purified with silica gel column chromatography, the substance was obtained as crystallized colorless platelets and chemically identified as the antifungal agent Valinomycin. The antitumor activity of Valinomycin was reported by A. J. Repta in 1981 as a result of the NCI screening test and was found to be effective against P-388 tumor. In an antitumor activity test carried out in our laboratory in vitro or/and in vivo, No. 22 substance was also revealed to be effective against P-388 tumor in host mice, KB cells, and so on.
The important two-dimensional echocardiographic finding of dissecting aneurysm in the acute stage is characterized by the presence of an oscillating intimal flap which is thought to be of highly diagnostic value. This report describes about three cases with dissecting aneurysm in which an oscillating flap was transiently observed. In Case 1 (62-year-old female), an oscillating flap observed in the aortic arch seven hours after the onset was not detected three days later. A flap in Case 2 (65-year-old male) which had been present in the descending aorta three hours after the onset of illness disappeared two days later. In Case 3 (55-year-old male), only an intimal flap without fine oscillation was demonstrated in the abdominal aorta by echocardiography performed three days after the onset of illness. In the acute phase of dissection, the echocardiographic detection of an oscillating flap seems to depend on the time of the study after the attack.
A case of adrenoleukodystrophy was studied morphologically and biochemically. The patient was a 28-year-old man with no family history of adrenoleukodystrophy. His neurologic symptoms were cerebellar ataxia, spastic paraplegia, pseudo-bulbar palsy, and a minimal visual disturbance, with neither adrenal nor hypogonadal symptoms. The morphological and biochemical findings in this case are identical with those in typical adrenoleukodystrophy, but the topographical distribution of the lesions is distinctly different. The changes selectively affect the white matter in the cerebellum and brain stem in contrast to minimal involvement of the occipital white matter.
Two Japanese quail which were incapable of wing movement and three normal quail were examined by histological and ultrastructural methods. The diseased birds had glycogen deposits in their skeletal muscle, cardiac muscle, smooth muscle, and nerve cells of the brain and spinal cord. According to the distribution of the lesions and the characteristics of the deposited glycogen, the diseased birds had glycogenosis which was analogous to type II found in man. The usefulness of this disease as a model for glycogenosis in man is discussed.
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A 51-year-old woman was admitted to our hospital because of palpitation, dyspnea and anterior chest discomfort. She had had a heart murmur since childhood. On physical examination, a rough grade 6/6 ejection systolic murmur was maximally audible at the second left intercostal space with a systolic thrill. The chest X-ray film showed the cardiothoracic ratio of 54% and the electrocardiogram demonstrated right ventricular hypertrophy. Calcification of the tricuspid annulus was recognized by chest X-ray films (anteroposterior and lateral views), M-mode and cross-sectional echocardiograms and the computed tomogram (CT) of the chest. Cardiac catheterization revealed the presence of valvular pulmonary stenosis and atrial septal defect. These findings were confirmed by surgical operation. Since the first description of Paktovskii in 1960, nine cases of calcification of the tricuspid annulus were reported, but no case has been described in the Japanese literature. Contrary to the mitral annulus, the echocardiographic and computed tomographic features of the calcification of the tricuspid annulus has not been reported previously. In this paper, we reported a middle aged patient with circumferential calcification of the tricuspid annulus in association with severe pulmonary stenosis and atrial septal defect, and the features of the echocardiogram and CT images were illustrated.
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Terminal deoxynucleotidyl transferase (TdT, EC 2.7.7.31) has been purified 4365-fold from pig thymus. It was further separated into two molecular forms of 57,000 and 45,000 dalton by Sephadex G-100 gel-filtration. The former sedimented at 4.2s through a sucrose gradient, while the latter, at 3.6s. By a SDS-polyacrylamide gel-electrophoresis, their molecular weights were estimated as 57,000 and 42,000 dalton, respectively. Thus each of the large and small pig TdT consists of a single polypeptide of 57,000 or 42,000 dalton and has no subunit structure. These two forms were indistinguishable in antigenicity by a neutralization assay of 42,000 dalton-TdT antibody. The enzymological properties of 42,000 dalton-TdT from pig thymus were very similar to those of calf TdT which has a two-subunits structure.
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Terminal deoxynucleotidyl transferase [EC 2.7.7.31] has been purified 4,365-fold from pig thymus. It was further separated into two molecular forms of 57,000 and 45,000 daltons by Sephadex G-100 gel-filtration. The former sedimented at 4.2S through a sucrose gradient, while the latter sedimented at 3.6S. By sodium dodecyl sulfate-polyacrylamide gel-electrophoresis, their molecular weights were estimated at 57,000 and 42,000 daltons, respectively. Thus, the large and small pig terminal deoxynucleotidyl transferases both consist of a single polypeptide of 57,000 and 42,000 daltons and have no subunit structure. These two forms were indistinguishable in antigenicity as examined by a neutralization assay with an anti-calf terminal deoxynucleotidyl transferase antibody. The enzymatic properties of 42,000-dalton terminal deoxynucleotidyl transferase from pig thymus were very similar to those of the calf enzyme, which has a two-subunit structure.
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