[The biological role of carnosine in excitable tissues]. / Biologicheskaia rol' karnozina v vozbudimykh tkaniakh.

The authors summarize the data about characters of histidine-containing dipeptides (HCD) carnosine and homocarnosine in excitable tissues. The value of their antiradical, pH-buffer and metal-chelating characters is presented. The authors analyze data on participation of some cell membrane receptors in biological effects of HCD. These results were used earlier as the evidence of mediator function of carnosine in centre nerve system, though later it was not supported experimentally. It is considered now that the biological role of HCD in tissue is connected with the neutralization of superfluous protons, metal ions of changeable valence and free radical compounds, so called general buffer action. The result of this action is the normalization of cell metabolism and the protection of membrane of excitable cells against negative environmental influences.

[Factors affecting the concentration of serum ceruloplasmin in Wilson-Konovalov disease]. / Faktory, vliiaiushchie na kontsentratsiiu syvorotochnogo tseruloplazmina pri bolezni Vil'sona-Konovalova.

An oxidase activity of ceruloplasmin was studied in blood serum of 335 patients with Wilson-Konovalov disease before specific copper-obviating treatment. Coefficient for calculation of concentration units depending on optic density was estimated by means of copper content measurement in ceruloplasmin. The well-known fact of ceruloplasmin content decrease in the Wilson-Konovalov disease was supplemented by the new data involving age-sex differences and the age-dependent dynamics as compared with healthy persons and with a group of 827 patients with other neurological diseases. Under conditions of the pathology concentration of ceruloplasmin was minimal in teenagers and in men it was lower than in women (62.1 +/- 4.0% and 88.2 +/- 5.0%, respectively, P < 0.001). Statistically distinct differences in content of ceruloplasmin were found in the patients of 15-19 years old and 20-24 years old as compared with the older age groups. Higher content of ceruloplasmin, exceeding the normal level about 200 mg/l, was most often detected at the preneurological step of the disease as compared with the neurological period (8.8% and 2.3%, respectively).

[Changes in the internal organs in the preneurological stage of hepatocerebral dystrophy]. / Izmeneniia vnutrennikh organov v prenevrologicheskoi stadii gepatotserebral'noi distrofii.

In the early stages (preclinical and visceral) of hepatocerebral dystrophy (HCD) where the liver gets damaged with no neurological manifestations, the patients usually turn to the internists, pediatricians, endocrinologists and psychiatrists. Familiarization with risk factors in respect to HCD appreciably facilitates the diagnosis, which is of paramount importance today since we dispose of the effective pathogenetic methods of its treatment. The early diagnosis of this hereditary disease makes it possible to examine the risk group (the patient's brothers and sisters) and to commence the preventive treatment.

[Protective effect rendered by human ceruloplasmin on erythrocytes in hepatocerebral dystrophy]. / Zashchitnoe deistvie, okazyvaemoe tseruloplazminom chekoveka na éritrotsity pri gepatotserebral'noi distrofii.

In order to elucidate the protective effect of human ceruloplasmin (CP) on erythrocytes in patients with hepatocerebral dystrophy (HCD), the parameters reflecting the interaction of CP from the blood of healthy donors (n-CP) and of HCD patients (h-CP) with erythrocytes from healthy donors (n-ER) and from HCD patients (h-ER) were estimated. The protective effects of n-CP and h-CP on n-ER and h-ER during the Cu2+-induced lysis were compared. It was shown that the ability of h-CP to prevent the human ER breakdown upon Cu2+-induced lysis is much lower (approximately 3-fold) than that of n-CP. The differences in the protective effect of n-CP and h-CP are manifested in a greater degree during the n-ER lysis than during the h-ER lysis.

[Analysis of the composition of the microelements in the hair of normal subjects and in disseminated sclerosis]. / Analiz sostava mikroélementov volos v norme i pri rasseiannom skleroze.

The modification of analysis of the hair trace elements involves hair dissolution in nitric acid and analysis of the iron, manganese, and copper by flameless atomic-absorption spectrometry and of zinc by the flame technique. Trace element content has been measured in the hair of 26 normal subjects aged 20-50 and of 20 children. In adults iron, manganese, zinc, and copper concentrations were 12.2 +/- 0.6, 0.55 +/- 0.11, 172 +/- 8, and 12.8 +/- 0.6 micrograms/g, respectively. Higher concentrations of manganese have been observed in females, as well as a tendency to an increase of zinc levels in adults. Analysis of copper in the hair of 44 patients with disseminated sclerosis, carried out by the suggested technique and by direct analysis of the solid sample in a graphite cuvette, has revealed a trend to an elevation of copper content vs. the norm. No significant differences between the hair manganese levels of females and males have been revealed in the patients, such differences being typical of normal subjects. Fluctuations in copper concentrations at different sites of a single hair and a strand have been analyzed in the patients and in normal subjects.

[Ceruloplasmin activity in the blood during progressive diseases of the central nervous system]. / Aktivnost' tseruloplazmina syvorotki krovi pri progressiruiushchikh zabolevaniiakh tsentral'noi nervnoi sistemy.

Ceruloplasmin (CP) preparations have been used as the reference agents in transition of this protein oxidase activity to blood serum concentrations; the mean concentrations of serum CP varied from 175 to 310 mg/l, depending on the biochemical characteristics of the preparations. Adjustment by the blood serum copper level has proved adequate for clinical studies. CP activity has been found increased in some patients with disseminated sclerosis and lateral amyotrophic sclerosis. CP activity grows with age in normal subjects and in the patients with hereditary extrapyramidal and cerebellar diseases and phenocopies thereof (except hepatocerebral dystrophy). CP activity is higher in women than in men.

[Changes in zinc metabolism during long-term D-penicillamine treatment of patients with Wilson-Konovalov disease]. / Izmenenie obmena tsinka na fone dlitel'nogo lecheniia D-penitsillaminom u bol'nykh bolezn'iu Vil'sona--Konovalova.

Using atomic absorption spectrophotometry, the authots determined zink levels (in correlation with the time-course of copper elimination) in the blood serum and urine of 28 patients with hepatocerebral dystrophy prior to and during the first year of D-penicillamine treatment and in 55 patients who had been on this therapy for a number of years. The zink concentration in the patients' blood serum prior to treatment tended to decline. Such a decrease was also noted in cirrhosis of other etiologies. Thiol therapy resulted in a gradual increase in the urine zink elimination which followed a pattern of the drug dose increment and could be two to three times higher than normal in cases of prolonged treatment. Nevertheless, these patients exhibited no symptoms of zink insufficiency and the zink concentration in the blood serum had been normal for a number of years, which may be explained by an enhanced absorption of zink from the intestines and by its mobilization from the tissue depots. The pathological manifestations observed in six patients with the asthenoneurotic syndrome regressed following zink administration.

[Metabolic disorders in essential tremor]. / Ob obmennykh narusheniiakh pri éssentsial'nom tremore.

Various parameters of the carbohydrate, lipid, protein, purine, and trace element metabolism were examined in 82 patients with essential tremor. Hypercholesterolemia, varying changes of the carbohydrate metabolism, lowered uric acid levels in the serum and the urine, and a tendency to a rise of the serum transferrin level were disclosed. The results obtained ae discussed in comparison with metabolic changes in other extrapyramid diseases.

[Metabolic disorders in Huntington's chorea]. / O metabolicheskikh narusheniiakh pri khoree Gentingtona.

The results of biochemical studies conducted in 42 patients with Huntington's chorea are presented. The authors marked the decrease of tolerance to glucose, the increase of cholesterol concentration in the blood serum in some patients. Uric acid concentration in the blood serum and its excretion with urine were decreased in all examined patients. Ceruloplasmic activity in the patients' blood did not differ from the normal level. Cuprum concentration in the blood serum and the patients' urine was within the normal fluctuations.