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Biomedical subjects

H Fujii

Publications and source records attributed to H Fujii.

At least 1,207 records · Page 67Linked to original sources
PubMed1980

Multiple components of blood group A and B antigens in human erythrocyte membranes and their difference between A1 and A2 status.

Human type O erythrocyte membranes were converted to type A1 by purified human A1-enzyme, to type A2 by purified human A2-enzyme, and to type B by purified human B-enzyme in the presence of radioactive sugar donors (i.e., UDP-N-acetylgalactosamine for A-enzyme and UDP-galactose for B-enzyme, respectively). Type A2 erythrocyte membranes were also converted to type A1 by purified A1-enzyme A1-enzyme. The labeled blood group antigens (A1, A2, and B) thus produced were analyzed by sodium dodecyl sulfate gel electrophoresis and by isoelectric focusing. It was demonstrated that blood group antigens on erythrocyte membranes are mostly (greater than 80%) glycolipid, not glycoprotein. The blood group glycolipids were presumably conjugated with other membrane materials and formed three major components in type A1 and type B erythrocyte membranes. However, two of the three components were absent in type O erythrocyte membranes converted to type A2. These results indicate the heterogeneity of blood group components in erythrocytes membranes and the qualitative difference between A1 and A2 antigens on these membranes.

ABO Blood-Group System↗
PubMed1980

Molecular abnormality of phosphoglycerate kinase-Uppsala associated with chronic nonspherocytic hemolytic anemia.

Inherited deficiency of phosphoglycerate kinase (PGK; ATP:3-phosphoglycerate 1-phosphotransferase, EC 2.7.2.3) is associated with chronic nonspherocytic hemolytic anemia and mental disorders in man. One such variant, PGK-Uppsala, was purified to homogeneity. PGK-Uppsala had a lower-than-normal specific activity (30% of normal in the backward reaction and about 20% of normal in the forward reaction) and higher-than-normal Michaelis constants for ATP, ADP, 3-phosphoglycerate and 1,3-diphosphoglycerate. Peptide mapping analysis revealed that the structural abnormality of PGK-Uppsala is a single amino acid substitution from arginine to proline at the 206th position. Based on the known complete amino acid sequence of the normal human PGK and the three-dimensional model deduced from horse PGK, correlations between the structural and functional abnormalities of PGK-Uppsala are discussed. Structural abnormalities of PGK-II, which is an electrophoretic variant not associated with enzyme deficiency, and PGK-München, which is associated with enzyme deficiency and heat instability but not associated with hemolytic anemia, are also discussed.

Amino Acid Sequence↗
PubMed1980

Computed tomography of multicystic kidney.

A case of multicystic kidney diagnosed by computed tomography (CT) and confirmed at surgery is presented. On CT, the lesion appeared as an abnormal mass composed of numerous walled, low density areas of varying sizes consistent with the surgical finding of congenital multicystic kidney.

Female↗
PubMed1980

Seven pyruvate kinase variants characterized by the ICSH recommended methods.

Seven new red-cell pyruvate kinase (PK) variants were characterized by the methods recently recommended by the International Committee for Stanardization in Haematology. The cases were all true homozygote as evidenced by consanguineous marriages of the parents; all are Japanese. These variants were designated as PK Tokyo, PK Nagasaki, PK Sapporo, PK Maebashi, PK Itabashi, PK Fukushima and PK Aizu, respectively. Low substrate affinity (high K0.5S for phosphoenolpyruvate) and thermal instability appear to play major roles in causing defective enzyme function, resulting in chronic haemolytic anaemia. Product inhibition of PK by ATP may also play an additional role in causing haemolysis in more than half the cases.

Adolescent↗
PubMed1980

The delay time of tooth vibration signal recorded at facial skin surface.

The onset of a tooth vibration signal with a mini acceleration pickup attached to the skin on the forehead was delayed by 0.1788 ms on average after the actual tooth impact. When the signal was recorded at the zygomatic arch, the delay time was 0.1008 ms on average, which was significantly shorter than that obtained from the forehead (P less than 0.001). The difference due to a transposition of the vibrated tooth was also smaller (0.025 less than P less than 0.05) and had almost no effect on the intensities of the tooth flipping force. The delay time was even more prolonged when a poor frequency specification pickup and/or low pass filter were applied.

Adult↗
PubMed1980

Structure and function of normal and variant human phosphoglycerate kinase.

Complete amino acid sequence of normal human phosphoglycerate kinase (PGK) was determined. The enzyme consists of 417 amino acid residues with acetylserine at the NH2-terminal and isoleucine at the COOH-terminal. The structural abnormality of PGK-II, which is fairly common in Southern Pacific populations, is a single amino acid substitution from threonine in the normal enzyme to asparagine in the variant enzyme at the 352nd position. The substitution induced no change in the enzyme activity, but induced strong binding of the variant enzyme with citrate. The structural abnormality of PGK-München is a single amino acid substitution from aspartic acid in the normal enzyme to asparagine in the variant enzyme at the 267th position. PGK-München is associated with red cell enzyme deficiency (about 20% of normal), and substantial heat instability. Therefore, the negative charge of an aspartyl residue at the 267th position must play a role in maintaining the stability of the enzyme molecule. Possible mechanisms of hemolysis due to hereditary deficiency of PGK are discussed.

Amino Acid Sequence↗
PubMed1980

Pyrimidine 5'-nucleotidase deficiency: studies of five cases in two Japanese families.

Two new families with red cell pyrimidine 5'-nucleotidase (P5N) deficiencies were found in Japan. The enzyme activities were 4.8% in case 1 and 9.7% in case 2. The propositi showed characteristic hemolytic anemia with market basophilic stippling, increased reduced glutathione content and accumulation of pyrimidine nucleotides in the red cell. High Michaelis constants for cytidine monophosphate, normal thermostability, abnormal optimum pH in both cases, and normal electrophoretic mobility in case 1 and slower mobility in case 2 were shown, suggesting that the cause of P5N deficiency, at least in these cases, is due to the structural gene mutation.

5'-Nucleotidase↗
PubMed1980

Purification and properties of adenosine deaminase in normal and hereditary hemolytic anemia with increased red cell activity.

Red cell adenosine deaminase from normal subjects and from a patient with hereditary hemolytic anemia with a 40-fold increase in activity were purified using antibody affinity chromatography. The purified enzymes were completely homogeneous on sodium dodecyl sulfate-polyacrylamide gel electrophoresis. There were no differences in the molecular weight, specific activity, polyacrylamide gel electrophoresis, Michaelis constant for adenosine, inhibition constant for guanylurea, utilization of 2-deoxyadenosine, thermal stability, optimum pH, immunological reactivity, amino acid composition and tryptic peptide mapping. These results strongly suggest that increased red cell adenosine deaminase activity is caused by an overproduction of a structurally normal enzyme.

Adenosine Deaminase↗