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Biomedical subjects

T Kudoh

Publications and source records attributed to T Kudoh.

At least 127 records · Page 7Linked to original sources
PubMed1979

Identification of keratan sulfate in liver affected by Morquio syndrome.

Glycosaminoglycan content, composition and molecular weight were determined in liver obtained from a patient with Morquio syndrome (Mucopolysaccharidosis IV). There was about a four-fold increase in glycosaminoglycan content (as hexosamine) of the affected liver as compared to the control liver. The major glycosaminoglycan accumulated in the liver was keratan sulfate, which was not found in the control liver. Chondroitin sulfates, especially chondroitin 6-sulfate, were also increased. Heparan sulfate isolated from the liver of a patient with Morquio syndrome was structurally different to that from control liver, and the glycosaminoglycans from Morquio syndrome were of a much lower molecular weight than those from control.

Adolescent↗
PubMed1979

Sphingomyelinase activities in cultured skin fibroblasts from patients with Niemann-Pick Disease.

Sphingomyelinase activity in cultured skin fibroblasts from a fetus affected with infantile-type Niemann-Pick disease was 0.5% of control activity; the activities in cells from two patients with adult-type disease (Cases 2 and 3) were 5.0% and 59.0%. Sphingomyelinase activiy was separated into three peaks (I-III) by isoelectric focusing. The isoelectric points were 4.5, 4.9, and 5.2 for peaks I, II, and III, respectively. The three peaks in the Case 2 cells were drastically reduced; only a very small peak could be distinguished (pI of 4.7). On the other hand, three peaks were observed in the Case 3 cells. Peak I had a pI of 4.4, peak II a pI of 4.7, and peak III a pI of 5.2. Peak I was found at near normal level, but both peaks II and III were markedly reduced. Sphingomyelinase in the peak I fraction obtained from isoelectric focusing in Case 3 cells was found to have the same Km value as that in control cells.

Adult↗
PubMed1979

Age-dependent variations of lysosomal enzymes in human liver.

The specific activities, the Km values, and the elution patterns on DEAE 52 and Sephadex G-150 columns of six lysosomal enzymes in human liver during development were studied. The levels of total beta-D-glucuronidase and N-acetyl-beta-D-glucosaminidase activities in childhood liver were higher than those in fetal liver. The Km values of beta-D-glucuronidase and beta-D-galactosidase in fetal liver were about ten times higher than those in childhood liver. The elution patterns on DEAE 52 of beta-D-glucuronidase and on Sephadex G-150 of alpha-D- and beta-D-galactosidases changed with aging. It is suggested that lysosomal enzymes related to degradation of gangliosides and glycosaminoglycans change during development.

Acetylglucosaminidase↗
PubMed1978

Prenatal diagnosis of GM1-gangliosidosis: biochemical manifestations in fetal tissues.

A prenatal diagnosis of GM1-gangliosidosis was made in a pregnancy at risk, on the basis of a deficiency of beta-galactosidase activity demonstrated in cultured amniotic fluid cells. Biochemical analyses were performed in the aborted fetus. GM1-ganglioside beta-galactosidase activity was reduced to 1% of the control value in both the brain and liver of the affected fetus. Lamellar bodies suggestive of membranous cytoplasmic bodies were found in cells of basal ganglions, while the accumulation of GM1-ganglioside in the brain was not remarkable.

Amniotic Fluid↗
PubMed1978

Lysosomal acid hydrolases in established lymphoblastoid cell lines, transformed by Epstein-Barr virus, from patients with genetic lysosomal storage diseases.

Lysosomal acid hydrolases were determined in established lymphoblastoid cell lines, transformed in vitro by Epstein-Barr virus (EBV) from lymphocyte-rich cell populations isolated from the peripheral blood of patients with genetic lysosomal storage diseases--Hurler syndrome, Scheie syndrome, GM1-gangliosidosis type 1 and type 2, Tay-Sachs disease, and I-cell disease--and from obligate heterozygotes for these diseases. The respective enzyme activity was undectectable in lymphoblastoid cells from the patients, but not from controls. Obligate heterozygotes could not always be distinguished from controls in lymphoblastoid cells as well as in leukocytes. These results suggest that established lymphoblastoid cell lines are useful material for the enzymatic study of genetic lysosomal storage diseases.

Cell Line↗
PubMed1978

Chronic Niemann-Pick disease with sphingomyelinase deficiency in two brothers with mental retardation.

Clinical, biochemical, and electron microscopic studies are presented in two brothers with Niemann-Pick disease. The clinical features include hepatosplenomegaly and mental retardation without any other neurological signs. Roentgenograms of the chest showed bilateral diffuse reticular infiltration. The amounts of sphingomyelin and cholesterol in liver were increased, and sphingomyelinase activities in both liver and skin fibroblasts were markedly reduced in Case 1. Numerous foam cells and myelin figures were observed in the liver, kidneys, bone marrow, and lymph nodes on electron microscopical examination. These cases were regarded as a variant of Niemann-Pick disease from our investigations as they have mental retardation as an exceptional symptom when they are diagnosed as type B.

Adolescent↗
PubMed1978

The abnormalities of beta-galactosidase in GM1-gangliosidoses.

The activity of GM1 beta-galactosidase in the brain and liver of patients with GM1-gangliosidosis was assayed using GM1-ganglioside tritiated in the terminal galactose. In the cases of GM1-gangliosidosis Types 1 and 2A the activity was less than 0.5% of the control. In the liver of GM1-gangliosidosis Type 2B the activity was observed to be much higher than that of Types 1 and 2A. On Sephadex G-150 gel filtration, three active fractions (I, II and III) for 4-methylumbelliferyl beta-galactopyranoside (4MU) and two active fractions (I and II) for GM1-ganglioside were obtained in the control liver. There was no active fraction for GM1-ganglioside in spite of the preserved fraction I for 4MU in the liver of GM1-gangliosidosis Type 1 or Type 2A. In any of the three cases fraction II for both 4MU and G71-ganglioside was not detected.

Brain↗
PubMed1977

alpha-L-Iduronidase activity in established lymphoblastoid cells from patients with Hurler and Scheie syndromes transformed by Epstein-Barr virus.

alpha-L-Iduronidase activity was determined in established lymphoblastoid cells, which were transformed in vitro by Epstein-Barr virus, of lymphocytes-rich cell populations isolated from peripheral blood of patients with Hurler and Scheie syndromes. alpha-L-Iduronidase activities in established lymphoblastoid cells from patients were undetectable, while activities of control subjects were clearly detected. These results suggest that established lymphoblastoid cells are useful for the enzymatic study of genetic mucopolysaccharidoses.

Adolescent↗
PubMed1976

Three cases of GM1-gangliosidosis.

A biochemical analysis was carried out on three cases of GM1-gangliosidosis which showed different clinical manifestations. These cases were classified in a previous study as Type 1, Type 2 (2B) and Type 2 (2A), an intermediate type between classical Type 1 and Type 2 (2B), by the determination of the chromatographic profile of the liver beta-galactosidase activities. Gangliosides, neutral glycolipids; phospholipids and glycopeptides were analyzed in the brain and the liver of these cases. The concentration of total ganglioside was increased in the brain in all cases. The elevation was due to an increase of GM1-ganglioside, which accounted for 63% or more of the total ganglioside, while in the control brain about 20% of the total ganglioside was GM1-ganglioside. In type 2A, increases of GM1-ganglioside and and asialo-GM1 in the liver were more prominent than those in the liver of Type 2B. The non-dialyzable glycopeptides were analyzed only in Type 2A. In the liver of Type 2A, the hexosamine and hexose contents of the non-dialyzable glycopeptides were about 10 times and 5 times higher than those of the control. These biochemical analyses revealed that Type 2A had intermediate characteristics between two Types. In this classification of the three Types, biochemical data were well correlated with clinical features.

Brain↗
PubMed1974

A simple technique for culturing tubercle bacilli.

Microscopy, traditionally used in peripheral health centres to diagnose tuberculosis, could be supplemented by sputum culture if sputum specimens were inoculated on suitable media and sent to intermediate or central laboratories to be incubated and read. To facilitate this procedure, the authors propose a simplified swab culture method and a modification of Ogawa's egg medium.

Bacteriological Techniques↗
PubMedDate not supplied

Periduodenal panniculitis due to spontaneous rupture of a pancreatic pseudocyst into the duodenum.

We report a patient with transient periduodenal panniculitis due to spontaneous rupture of a pancreatic pseudocyst into the duodenum. He developed sudden onset of severe epigastric and back pain with jaundice, mimicking the symptoms of acute pancreatitis. However, the serum and urinary amylase levels did not increase. CT scans showed disappearance of his pseudocyst and periduodenal panniculitis without any evidence of acute pancreatitis. The CT findings of periduodenal panniculitis and his symptoms both improved within 3 weeks. A duodenal fistula leading to the remnant pseudocyst and narrowing of the periduodenal portion of the common bile duct were demonstrated by endoscopic retrograde cholangiopancreatography.

Aged↗