[Invariable chain (Ii-chain) as a highly important molecular chaperone of histocompatibility class II molecules]. / Invariabel'naia tsep' (Ii-chain) kak vazhneishii moleculiarnyi shaperon molekul gistosovmestimosti classa II.

The publications on the structural and functional features of the main molecular shaperon of li-chain were updated. In the 1990s the determination of molecular shaperon as a group of mutually unrelated protein molecules taking part in the assembly of other polypeptides was worked out. In humans the main isoform of li-chain is the protein with a mol. wt. of 31-33 kD. A great variety of functions of this shaperon is linked with definite amino acid sequences. In particular, the molecular shaperon of the molecules of the main histocompatibility complex-II has functions connected with the presentation of antigen and the differentiation of B lymphocytes.

[Potentialities of calcium dependent lectin receptors of antigen presenting cells for the immunotherapeutic preparation development]. / Vozmozhnosti ispol'zovaniia kal'tsii-zavisimykh lektinovykh retseptorov antigen-prezentiruiushchikh kletok glia sozdaniia immuno-terapevticheskikh preparatov.

Publications on C-lectin receptors, multifunctional surface molecules of antigen presenting cells are updated. The study of C-lectin receptors is stipulated by their role in antimicrobial protection. The importance of C-lectin receptors of dendrite cells in the system of the recognition of microbial carbohydrate as well as their properties of the targeted supply of tumor cell and other antigens to dendrite cells with the purpose of the formation of immunity against faintly immunogenic antigens is considered. Suggestion is made on the potential of their use as experimental preparations for immunotherapy.

[Comparative study of collagen in hypertrophic and keloid cicatrix]. / Sravnitel'noe issledovanie kollagena gipertroficheskikh i keloidnykh rubtsov.

The relative percentage of type III to type I collagen, the ratio of alpha 1(I) to alpha 2(I) collagen chains and the ratio of dimers (beta-chains) to monomers (alpha-chains) in type I collagen have been measured in solubilized collagen fractions from keloids and hypertrophic scars aged about 1-7 years after burn. In tissue samples the content of crosslink with structure of pyridinoline were analyzed and expressed as mole of crosslink per mole of collagen. A comparison between hypertrophic and keloid scars has shown that the young (about 1 year) hypertrophic scars have higher ratios alpha 1(I)/alpha 2(I) and beta 11 + beta 12/alpha 1(I) + alpha 2(I). The increased proportion of beta-chains in the younger hypertrophic scars approximated to the same level as in normal skin within 7 years after lesion. There was no decrease in the ratio dimers/monomers of collagen type I with age of keloid scar tissue. The relative amount of collagen type III in young keloid scars was found decreased as compared to age-matched hypertrophic scars (13 +/- 3 and 17 +/- 7 respectively), but was similar in the older scars. The average pyridinoline crosslinks content per mole of collagen in keloids was 2 times as high as in hypertrophic scars. The data support the suggestion that general, as well as peculiar disturbances of collagen metabolism are involved in the development of keloids and hypertrophic scars.

[Collagen metabolism in hypertrophic and keloid scars]. / Issledovanie metabolizma kollagena gipertroficheskikh i keloidnykh rubtsov.

Imbalance between the synthesis and degradation of collagen is a common sign of tissues of hypertrophic and cheloid scars. The fact that the collagen of abnormal scars of the both types have a polyfunctional linkage with a structure of pyridinol and high amounts of a polyfunctional linkage with a structure of pyrrole (Ehlich's chromogen) in the collagen of cheloid scars support the assumption that the collagen synthesis-degradation imbalance may be due to the enhanced resistance of structurally unchanged collagen to collagenolytic enzymes. The comparative studies of the ratio of type 1 collagen homotrimers to heterotrimers, that of type 1 collagen dimers to monomers, and that of types 1 to type 3 collagen in the pepsin extracts of abnormal scars of various age and patients' intact skin suggest that there are certain differences in the magnitude and scar age-related changes in the expression of collagen genes and posttranslational collagen modifications in the tissue of hypertrophic and cheloid scars. It is suggested that age-specific time course of collagen metabolic and structural changes in the abnormal scars may result from their alterations of the population composition of fibroblasts with a different phenotype.

[Age changes in the level of pyridinoline cross-linking and ratio of soluble and insoluble collagen in human rib collagen]. / Vozrastnye izmeneniia soderzhaniia piridinolinovykh sshivok i otnosheniia rastvorimogo i nerastvorimogo kollagena v rebernom khriashche cheloveka.

The changes in the content of mature crosslinks with pyridinoline structure and soluble/insoluble collagen ratio in the costal cartilage tissue of human beings aged from 1 month to 57 years were found to be age-dependent. The effect of the pyridinoline crosslink content on the soluble/insoluble collagen ratio in human costal cartilage tissue may constitute no less than 67% of the total influence of the sum of all factors. The pronounced nonlinearity of the studied dependencies points to a possible involvement of a factor(s) other than the pyridinoline crosslink content.

[Comparative characteristics of oxyproline excretion in urine in children with post-burn hypertrophic and keloid scars]. / Sravnitel'naia kharakteristika ékskretsii oksiprolina s mochoi u detei s posleozhogovymi gipertroficheskimi i keloidnymi rubtsami.

Catabolism of collagen was studied in 25 children with keloid and hypertrophic scars by means of evaluation of total and polypeptide-bound hydroxyproline excreted with urine. Formation of postburn pathological scars led to an increase in excretion of collagen degradation products, the rate of the increase depended on area and age of scar. In hypertrophic cicatrization rate of collagen metabolites excretion correlated with the scar age reversely, demonstrating the tendency to decrease of the scar collagen metabolites with its ageing. Excretion of collagen metabolites with urine in patients with keloid scars depended on area of the scar and only slightly on its age. Besides, in patients with keloids rather high, statistically distinct correlation (r = 0.855) was found between the ratio macrophages/neutrophils under studies using "skin window" as well as between the ratio of hydroxyproline content in the polypeptide fractions with molecular mass above 1,500 and in the fraction containing oligopeptides and free hydroxyproline. This correlation was not found in patients with hypertrophic scars. Interaction of macrophages, neutrophils and, probably, eosinophiles appears to have a definite importance in regulation of collagen degradation, impairment of which may be substantial for scars formation.

[The connection between the decomposition of mature collagen and the level of natural cytotoxicity in patients with focal scleroderma]. / Sviaz' mezhdu raspadom zrelogo kollagena i urovnem estestvennoi tsitotoksichnosti u bol'nykh ochagovoi sklerodermiei.

Increased rate of pyridinoline excretion with urine was detected in patients with focal sclerodermia: the ratio pyridinoline/creatinine was equal to 0.094 +/- 0.049 as compared with that of 0.073 +/- 0.049 in controls. Concentration of mature collagen cross-links involving pyridinoline structure in urine of patients with focal sclerodermia did not depend on the patients age and the disease duration. However, this pattern correlated distinctly with clinical form of the disease (0.073 +/- 0.049 in local form and 0.013 +/- 0.017 in generalized form of sclerodermia) as well as correlated reversely with the rate of activity of natural killer cells (r = -0.977).

[Excretion of pyridinoline with urine in children with Ehlers-Danlos syndrome and Marfan's syndrome and the effect of beta blocker and complex vitamin therapy on it]. / Ekskretsiia piridinolina s mochoi u detei s sindromami Elersa-Danlo i Marfana i vliianie na nee terapii beta-adrenoblokatorom i kompleksom vitaminov.

Excretion of pyridinoline and polypeptide-bound hydroxyproline with urine was studied in 27 children with hereditary impairment of connective tissue. At the same time, effects of beta-adrenoblocking agents and vitamin complex, prescribed during preoperation period before thoracoplasty in hereditary chest deformation, were investigated. Clinical efficiency of the treatment depended distinctly on the initial value of ratios pyridinoline/creatinine and polypeptide-bound hydroxyproline/creatinine. Total positive effect of the therapeutic course, considering also echocardiographic examination and postoperational complications, was observed in 44% of patients with Ehlers-Dunlop syndrome and in 75% of patients with Marfan syndrome.

[Physiological changes in pyridinoline excretion in urine depending on age and sex]. / Fiziologicheskie izmeneniia ékskretsii piridinolina s mochoi v zavisimosti ot vozrasta i pola.

Natural physiological alterations in excretion of pyridoline (total hydroxypyridoline and lysyl pyridoline) with urine were studied in children and adults depending on age (1-86 years old) and sex. Age-dependent alterations of pyridoline excretion with urine had apparently four phases: an increase, a decrease, stabilization and secondary increase. Distinct dissimilarity between men and women in the rate of pyridoline excretion was found only in the age group of 16-21 years old.

[The protective activity of polyclonal and monoclonal antibodies to the lipopolysaccharide of Neisseria meningitidis serogroup A in in vivo experiments]. / Protektivnaia aktivnost' poliklonal'nykh i monoklonal'nykh antitel k lipopolisakharidu Neisseria meningitidis serogruppy A v opytakh in vivo.

The protective activity of the sera of mice immunized with the preparations of native and detoxified N. meningitidis lipopolysaccharide (LPS), group A, as well as with monoclonal antibodies to N. meningitidis antigens, groups A and B, was studied on the mucin model of meningococcal infection. The study showed that the maximum level of anti-LPS antibodies in mice was observed on day 7 after the injection of LPS. Immune sera obtained from mice were capable of protecting the animals from fetal meningococcemia induced by N. meningitidis strains of homologous and heterologous groups. As shown by the results of this study, the alkaline treatment of N. meningitidis native LPS did not decrease the protective properties of antibodies. The monoclonal antibodies under study were found to possess high preventive activity in mice challenged with N. meningitidis, groups A and B. Anti-LPS monoclonal antibodies showed greater protective activity than antipolysaccharide monoclonal antibodies.

[Contribution of lysosomal enzymes into degradation of short- and long-lived proteins in human embryonal fibroblasts with abnormal chromosome complement]. / Issledovanie vklada lizosomal'nykh fermentov v degradatsiiu korotko- i dolgozhivushchikh belkov émbrional'nykh fibroblastov cheloveka s anomal'nym naborom khromosom.

Degradation of short- and long-life proteins was studied in human embryonal fibroblasts with normal karyotype, trisomy and triploidy. Degradation of both short- and long-life proteins by means of lysosomal enzymes was elevated in fibroblasts with anomalous karyotype at the logarithmic phase of growth as compared with normal state, when lysosomotropic agent NH4Cl was used. In transition to stationary phase of growth participation of the lysosomal system in degradation of long-life proteins was increased in fibroblasts with normal karyotype, whereas degradation of these proteins in aneuploid fibroblasts maintained at the level found during the logarithmic phase of growth. Lysosomal enzymes did not participate apparently in degradation of short-life proteins in fibroblasts with normal and anomalous karyotype at both phases of growth. The data obtained suggest that chromosomal disbalance was related to impaired regulation of protein degradation as well as to possible alteration in the ratio of cytosol and lysosomal proteolysis.

[The effect of ascorbic acid on the collagen synthesis in skin fibroblasts of children with funnel chest]. / Vliianie askorbinovoi kisloty na sintez kollagena fibroblastami iz kozhi detei s voronkoobraznoi deformatsiei grudnoi kletki.

Relative synthesis of collagen was studied in skin fibroblasts of children with funnel chest and of corresponding age children (control) in presence of ascorbic acid. In presence of ascorbic acid the rate of collagen synthesis was 2-2.4-fold lower that in corresponding controls both in proliferating and stationary cultures. At the same time, relative synthesis of collagen was quite similar both in the patient and control fibroblasts in presence of ascorbic acid. Estimation of the 14C-Hypro/14C-Pro ratio in collagens isolated from cultural media showed that there was no difference in the hydroxylation rate of collagens in control and patient fibroblasts. These data suggest that funnel chest is one of the forms of systemic connective tissue diseases, which impaired mainly the cartilages.

[The effect of collagen-normalizing therapy on the incidence and severity of anesthetic and postoperative complications in children with connective tissue syndromes]. / Vliianie kollagenonormalizuiushchei terapii na chastotu i tiazhest' anestesiologicheskikh i posleoperatsionnykh oslozhenii u detei s soedinitel'notkannymi sindromami.

A new method of collagen-normalizing therapy has been worked out to prevent specific anesthesia-induced, operative and postoperative complications and to treat some symptoms in children with Ehlers-Danlos and Marfan's syndromes and with non-classified Marfan-like malformations. The technique involves a combined use of beta-adrenoblocker in the age-matched doses and vitamins C/0.03 g/(kg.day)/, B2/0.0004 g/(kg.day)/and B6/0.002 g/(kg.day)/ for 2.5 months before surgery and during the first 2 weeks of the postoperative period. The above therapy reduces the incidence of delayed recovery of the muscular tone and adequate respiration, spontaneous and recurrent pneumothorax, hemorrhagic and gastroenterologic complications, as well as the incidence and severity of intestinal paresis. The indexes of effective collagen-normalizing therapy are as follows: body weight increase, echocardiographic pattern of reduced diameter of the aorta and mitral valve prolapse, normalization of the urinary excretion of total and polypeptide-bound oxyproline. The efficacy of therapy depends on the baseline level of oxyproline excretion.

[Structural characteristics of collagens from the skin and rib cartilage of patients with Ehlers-Danlos syndrome type II]. / Strukturnye kharakteristiki kollagenov kozhi i rebernogo khriashcha bol'nykh s sindromom Elersa-Danlo II tipa]

Collagens were analyzed in skin and rib cartilage of 9 patients with Ehlers-Danlos syndrome of the II type. Electrophoresis and CNBr-peptide mapping showed that extended inserts and deletions as well as rough impairments of post-translation processing were not detected in collagens of the I, II and III types from these patients. In the patients with Ehlers-Danlos syndrome of the II type distinct increase was observed both in the total ratio of collagens III/I (P = 0.95) and in the ratio of intact collagens III/I free of cross-links. A decrease in content of dimers beta 11 and beta 12 was found in two patients. The data obtained suggest that the Ehlers-Danlos syndrome of the II type involved deteriorations in the structure of collagens I responsible for decrease in stability and sometimes for impairments in cross-link formation. Increase in content of collagen II fraction, predisposed to proteolytic hydrolysis of terminal sites, as well as elevated sensitivity of collagen II to pepsin hydrolysis were found in collagens of rib cartilage from patients with the syndrome and with funnel chest deformation. This suggests the lowered stability of collagen II from rib cartilage in funnel chest deformation.

[Changes in the structure of type I collagen and cross-links between type I and type III collagen chains in a patient with funnel chest]. / Narushenie struktury kollagena I tipa i nalichie sshivok mezhdu tsepiami kollagenov I i III tipa u bol'nogo s izolirovannoi formoi kilevidnoi deformatsii grudnoi kletki.

Skin and rib cartilage collagens were studied in patient 1.I.K. with isolated form of pigeon chest as well as in a group of children without any impairments of connective tissue. Distinct decrease in stability of collagen I, an increase in the ration of alpha 1 (I)/alpha 2(I) chains and impairment in formation of beta 12 dimers were detected in the patient with pigeon chest. In the patient skin total ratio between collagens I and III, calculated from a content of BrCN-peptides, was similar to normal level, whereas the proportion was markedly increased between intact molecules of collagens III and I free of cross-links, which was calculated from the ratio of alpha 1(III)/alpha 2(I) chains. Presence of cross-links between alpha 1 (III) and alpha 2 (I) chains as well as between alpha 1 (III) and alpha 2 (I) chains was detected after peptide mapping of polypeptides arranged in the region of beta 11 and beta 12 dimers. All the collagen I preparation, extracted from skin of the patient 1.I.K., contained molecules with unstabilized N-terminal sites. These results suggest that mutation occurred in the N-terminal region of alpha 1(I) chain. Analysis of collagen from the patient 1.I.K. rib cartilage demonstrated a slight decrease in total stability of collagen II as well as elevated concentration of collagen II molecules containing unstabilized N-terminals. Mechanisms responsible for formation of cross-links between polypeptide chains of collagens I and III detected in human skin are discussed.

[The ratio of collagens type I and III in cultures of human fibroblasts with diploid and aberrant chromosome complement]. / Sootnoshenie kollagena tipov I i III v shtammakh fibroblastnykh kletok cheloveka s diploidnym i anomal'nym naborom khromosom.

The ratio of collagens I/III was studied in human diploid, trisomic and triploid fibroblast strains. The following material was used in experiments: 5 strains obtained from human spontaneous abortuses (trisomy 7, trisomy 9, triploidy), 6 diploid embryonic strains, 4 strains from patients with Down syndrome, 3 strains from healthy children, 4 strains from healthy adult donors and 3 strains from very old persons (81-107 years old). Various types of collagen were separated by means of electrophoresis. Distinct ontogenetic alterations were not observed in content of collagen III in diploid and aneuploid human cultivated fibroblasts. However, considerable alterations in the ratio of collagens I/III correlated with genetic pathologies only in the patients examined.