[Wide range of the use of natural lipases and esterases to inhibit Mycobacterium tuberculosis].

Lipases and/or esterases (hereinafter referred to as esterases) isolated from the wax moth (Galleria mellonella) were found to have a bacteriological action on Mycobacterium tuberculosis (MBT) H37Rv. Different types of raw esterase preparations (REP) were incubated with MBT at 37 degrees C for 18 hours, the incubate was seeded on the Finn-II solid medium or intraperitoneally injected into guinea pigs in a single dose of 100,000,000 bacteria. There was no growth of MBT in the medium within 8 weeks, some variants of REP causing a destruction of the medium for 3-7 days.

[Active form of dihydropteridine reductase in human chorion cells. Possibility of prenatal diagnosis].

Activity of dihydropteridine reductase was studied in human chorion cells for development of accurate methods for prenatal diagnosis of phenylketonuria (especially its most severe form known as lethal hyperphenylalaninemia).

[Oligomerization of phenylalanine hydroxylase during its activation with phenylalanine].

Activated and nonactivated forms of phenylalanine hydroxylase varied in the activity within the first minutes of the reaction initiated by means of a synthetic coenzyme 6,7-dimethyl-5,6,7,8-tetrahydropteridine. In activation of the enzyme by phenylalanine the degree of its oligomerization was altered. As shown by chromatography and ultracentrifugation nonactivated enzyme was a dimer with molecular mass 130,000 daltons, whereas the activated form was found to be tetramer of 260,000 daltons.

[Phenylketonuria and hyperphenylalaninemia: clinico-genetic classification of 14 forms].

The current classifications of the clinical patterns of phenylketonurias and hyperphenylalaninemias are reviewed. On the basis of the literature data, a new classification considering the type of the mutant gene, the severity of the biochemical defect and the disease clinical course is proposed. All 14 hereditary determined patterns are divided into three groups: 1) with complete or partial deficiency of the major enzymes of the reaction of oxidation of phenylalanine into tyrosine; 2) with defects of enzymes in the chain of the cofactor synthesis; 3) with enzymatic defects in conjugated links of phenylalanine and tyrosine metabolism.

[Dihydropteridine reductase activity in leukocytes and cultured fibroblasts of health individuals and of patients with the classical form of phenylketonuria].

Activity of dihydrohypteridine reductase, estimated by means of spectrophotometric method, was found in cell culture of human fibroblasts and in leukocytes of peripheric blood. The enzymatic activity was not altered markedly with time; thus suggesting a possibility to classify dihydropteridine reductase as a constitutive enzyme. In patients with typical form of phenylketonuria the enzymatic activity tended to decrease as compared with healthy persons.

[Use of modified Ayling's method for detection of homozygotes and heterozygotes for phenylketonuria gene].

Activity of phenylalanine hydroxylase from human leukocytes was shown to depend on concentration of phenylalanine and pteridine cofactor; optimal concentrations of the substances were estimated. Using this optimized procedure activity of phenylalanine hydroxylase was studied in leukocytes of homo- and heterozygotes by the phenylketonuria gene as well as in the cells of donors. The mean values of the enzymatic activity were distinctly different in the groups of homo-, heterozygote-bearing patients and in healthy persons, although a slight overlapping of the patterns was observed between the groups.

[Genetical heterogeneity of phenylketonuria].

Data on genetic nature of phenylketonuria molecular mechanisms of its pathogenesis and approaches to treatment and prophylaxis of the disease are reviewed. Genetic heterogeneity of phenylketonuria, dependent on polylocus control of phenylalanine hydroxylase complex, is considered in detail. A possibility is discussed of the existence of the genetically different forms of phenylketonuria.

[Presence of active phenylalanine hydroxylase in human leukocytes].

In homogenates of human leukocytes phenylalanine hydroxylase activity was shown to depend on concentration of protein, presence of specific hydroxylase inhibitors as well as on a dose of Ph+ gene; Km value was estimated for phenylalanine and cofactor. The data obtained suggest that human leukocytes contain membrane-bound phenylalanine hydroxylase, which is active in vitro and is similar apparently to the liver enzyme in its properties. Solubilization of the enzyme was achieved without alteration in its activity when 0.

[Changes in enzyme activities in early passages of cultured fibroblasts].

The specific activities and variability coefficients (Wn) of specific activities of seven enzymes (5 lines) of cultured embryonic human fibroblasts were studied. The experiments were carried out on the 2nd--6th passage (between 12th and 19th passages); in each passage simultaneously up to 3-10 vials collected on the 8th cultivation day were analyzed. According to the Wn value, the enzymes under study can be divided into two groups, i.

[Detection of phenylalanine hydroxylase activity in human leukocytes and fibroblasts].

The data obtained indicate the presence of phenylalanine hydroxylase activity in human leucocytes and fibroblasts. The following methods were used: estimation of accumulation of the oxidized form of the pteridine cofactor after Ayling and coworkers and radiochemical method. Probably this activity is connected with cell membranes and can be solubilized by treatment of cells by Tritone X-100.

[Effect of gene dose in human cells].

Data from literature are reviewed on the gene dosage effect in human cells. Until recently the dosage effects have been distinctly shown for the genes of superoxide dismutase-1, acid phosphatase of erythrocytes, adenine phosphoribosyl transferase, glutathione reductase and nucleoside phosphorylase, located on chromosomes 21, 2, 16, 8, 14, respectively. The data are discussed in the light of problems arising from the study of the regulation of gene expression in man.

Estimation of correlation of lactate dehydrogenase subunits mole quota based on differences in substrate inhibition.

A kinetic method of estimating the mole quota ratios of the human lactate dehydrogenase (LDH) H and M subunits based on differences in substrate inhibition of LDH isoenzymes by lactate is porposed. Stability of kinetic constants for a prolonged period of time is demonstrated. The dependence of the activity ratios on the contribution of the mole quota of the M-subunit of LDH is studied under conditions of low and high substrate concentrations.

[Changes in the mole fraction ratio of lactate dehydrogenase subunits in the lymphocytes of Down's syndrome patients].

A comparative study of the total activity and mole quota ratio of lactate dehydrogenase subunits in lymphocytes of 14 patients with Down's syndrome (trisomy-21) and in 10 healthy persons is carried out. Differences in the total activity in both groups were insignificant. In patients with Down's syndrome the mole quota ratio of H and M subunits of LDH was found to be significantly altered (p greater than 0.

[Comparative study of the changes in lactate dehydrogenase isoenzymatic spectra in the course of organogenesis in mice].

The dynamics of changes in the lactate dehydrogenase (LDH) spectra during organogenesis in CBA mice has been studied by means of ultramicroelectrophoresis. The embryonic period of development is characterized by the predominance of cathodic isozymes (LDH-5 and LDH-4) in all the organs under study. The increase of anodic isozymes (LDH-1 and LDH-2) takes place in the heart, kidneys and brain as the development proceeds.

[Dosage effect of the cytoplasmic superoxide dismutase (SOD-1) gene in the erythrocytes of Down's syndrome patients].

The activity of cytoplasmic superoxydase (SOD-1) was studied in erthrocytes of 17 patients affected with Down's syndrome (trisomy 21) and in 26 healthy persons. A 1.56-fold increase of the enzyme activity was observed in the group of patients as compared with the control group.

[Genetic heterogeneity of G6PD deficiency: mutant alleles of G6PD in the Shekii district of Azerbaijan].

Examination on G6PD deficiency in 349 patients of Shekii district hospital (Azerbaijan) revealed 16 hemi-, 4 homo- and 9 heterozygotic carriers of the defect. Gd- frequency, calculated from the data obtained (7.7%), may be compared to neighbouring regions' frequencies (6-30%).

[Use of differences in substrate inhibition for determination of the interrelationship between molar fractions of lactate dehydrogenase subunits].

A kinetic method of estimating the ratio of mole quota of H and M human lactate dehydrogenase (LDG) subunits is proposed, based on changes in substrate inhibition of LDG isoenzymes with lactate. Stability of kinetic constants for a long period of time is demonstrated. The dependency of activities ratio under low and high substrate concentration on the contribution of mole quota of LDG M subunits is studied.

[Modified method of ultramicroelectrophoretic protein fractionation].

A modification is described for the method of ultramicroelectrophoretic fractioning of proteins in polyacrylamide gel in capillaries 400--100 microns in diameter. The original construction of the assembly of apparatuses is offered which can be assembled from details made in the USSR. The operation of dosed filling of capillaries is significantly facilitated.