[Wilson-Konovalov disease in 3 sisters: a radical change in prognosis if timely diagnosed].

Wilson-Konovalov disease is a rare autosomal recessive genetic disorder in which copper accumulates in the liver, brain and other target organs. The paper describes a family case of the abdominal form of the disease in three sisters, the eldest of them died from fulminant liver failure at the age of 18 years. The second sister aged 16 years was diagnosed as having the disease at the stage of decompensated liver cirrhosis; her treatment with D-penicillamine resulted in complete disease remission. The youngest sister was diagnosed with the disease at the preclinical stage, which could expect its good prognosis. However, the patient's refusal of treatment led to death from liver failure. This case demonstrates the importance of timely diagnosis and the possibility of dramatic improvement in prognosis even at the stage of decompensated liver cirrhosis.

Effect of cholinergic drugs on the activity of basic carboxypeptidases in rat nervous tissue.

Effects of a single administration of cholinergic drugs (arecoline, atropine, nicotine, mecamylamine) on the activity of carboxypeptidase H and of phenylmethylsulfonyl fluoride-inhibited carboxypeptidase, which are involved in metabolism of neuropeptides, were studied in brain parts and the adrenal glands of rats. The enzyme activities were determined fluorimetrically using specific inhibitors and substrates. In the majority of cases the enzyme activities decreased, and this decrease was retained for at least 72 h. Changes in the activities of the studied enzymes depended on the type of cholinergic action, the nervous system part, and the time after the injection. The changes in activities of the studied carboxypeptidases are supposed to be a possible mechanism responsible for changes in the levels of neuropeptides under the influence of high doses of the drugs.

[Functional expression of bovine retina adenylate cyclase b fragment cDNA in Escherichia Coli cells]. / Funktsional'naia ekspressiia fragmenta kDNK guanilattsiklazy B setchatki byka v kletkakh Escherichia coli.

A 1488-bp fragment of bovine retina guanylate cyclase B gene encoding the catalytic and dimerizing domains as well as part of the protein kinase domain was expressed in Escherichia coli cells. The expression product was obtained as inclusion bodies and solubilized in 6 M guanidine hydrochloride. The fragment of guanylate cyclase B is a dimer close in catalytic activity to the native enzyme.

[Deletion mutants of the natriuretic peptide receptor type B: expression of cDNA, purification and characteristics of proteins]. / Deletsionnye mutanty natriureticheskogo peptidnogo retseptora tipa B: ékspressiia kDNK, ochistka i kharakteristika belkov.

The C type natriuretic peptide (CNP) is a peptide hormone stimulating vasorelaxation and inhibiting cell proliferation. CNP activates the type B natriuretic peptide receptor (NPR-B), known as the guanylate cyclase B membrane enzyme, which results in the cGMP release. To study functional properties of NPR-B, its gene fragments were expressed in methylotrophic yeasts Pichia pastoris. Conditions were found providing for secretion of functionally active recombinant proteins NPR-Bs and NPR-B1 into the cultural medium in a yield of 25 mg/ml culture. Their specific activity was 0.97 and 0.93 mumol cGMP min-1 mg-1 protein, respectively. It was shown that NPR-B belongs to the family of Ser/Thr protein kinases and can be autophosphorylated at the serine residues.

[Ras-like proteins of the invertebrate and vertebrate visual systems. Interaction of exoenzyme C3 substrates with photoactivated squid rhodopsin]. / Ras-podobnye belki zritel'noi sistemy bespozvonochnykh i pozvonochnykh. Vzaimodeistvie substratov ékzoénzima C3 s fotoaktivirovannym rodopsinom kal'mara.

Composition and cell localisation of ADP-ribosyltransferase C3 substrates were studied in squid photoreceptors and in bovine ROS. Polypeptides with M(r) 22, 24, 30, 45 and 80 kDa are ADP-ribosylated in squid photoreceptor membranes, and polypeptides with M(r) 22, 24, and 60 kDa are ribosylated in cattle ROS membranes. Cytoplasmic fraction of ROS contains C3 substrates with M(r) 22, 24, 28 and 36 kDa. Increase in ADP-ribosylation of 22 and 24 kDa substrates and their interaction with photoexcited rhodopsin in squid photoreceptors was found.

[Osmometry as a method of control of homeostasis in acute suppurative destructive diseases of the lung and pleura]. / Osmometriia kak metod kontrolia gomeostaza pri ostrykh gnoino-destruktivnykh zabolevaniiakh legkikh i plevry.

The osmolar status was studied in 63 patients with acute abscesses of lungs and empyema of pleura at different stages of the conservative and operative treatment. The hypoosmolarity syndrome was revealed at the acute stage of the disease against the background of a pronounced purulent intoxication. An increased excretion of nonidentified osmotically active metabolites with urine was registered at the height of pyodestructive process and postoperative disease.

[Intraoperative hemodilution during reconstructive surgery on the aortofemoral segment]. / Intraoperatsionnaia gemodiliutsiia pri rekonstruktivnykh operatsiiakh na aortobedrennom segmente.

The authors analyze results of using the intraoperative hemodilution with the help of the mannite plasma in 20 patients with obliterating lesions of the lower extremity vessels. Its influence on the central hemodynamics, water-electrolyte homeostasis, functions of the kidneys, liver, coagulating blood system were studied. The advantages of such method of autohemotransfusion are shown.

Purification of plant protein phosphatase PP7 and evidence for its redox regulation.

PP7, a recently identified protein Ser/Thr phosphatase of the PPP family distantly related to phosphatases PP5/PPT and PPEF/rdgC, was purified from cauliflower extracts to apparent homogeneity. Purified cauliflower PP7 and recombinant PP7 expressed in Escherichia coli exhibit light absorption in the visible range with a maximum at approximately 430 nm. Under nonreducing conditions, native PP7 exists as a mixture of monomer with an intramolecular disulfide bridge, disulfide-linked homodimer, and possibly disulfide-linked complexes with potential partner proteins. The activity of recombinant Arabidopsis thaliana PP7 is reversibly regulated by redox agents. The results demonstrate the existence of PP7 protein in planta and suggest a possibility of redox regulation of this protein phosphatase.