Congenital nephrotic syndrome in mice lacking CD2-associated protein.

CD2-associated protein (CD2AP) is an 80-kilodalton protein that is critical for stabilizing contacts between T cells and antigen-presenting cells. In CD2AP-deficient mice, immune function was compromised, but the mice died at 6 to 7 weeks of age from renal failure. In the kidney, CD2AP was expressed primarily in glomerular epithelial cells. Knockout mice exhibited defects in epithelial cell foot processes, accompanied by mesangial cell hyperplasia and extracellular matrix deposition. Supporting a role for CD2AP in the specialized cell junction known as the slit diaphragm, CD2AP associated with nephrin, the primary component of the slit diaphragm.

[Changes in the peripheral blood lymphocytes after immunization and its effect on the course of experimental inflammatory process in the lungs]. / Izmeneniia leikotsitov perifericeskoi krovi pri immunizatsii i ee vliianie na techenie eksperimental'nogo vospalitel'nogo protsessa v legkikh.

It was shown that in the immunization of rabbits with sorbed staphylococcus toxoid and human albumin, at the height of the immune response there occurred an intensification of biosynthesis and destabilization of lysosomal membranes of blood leukocytes, with a simultaneous development of microcirculation disturbances in the lungs. Development of pneumonia against the background of immunization led to a greater destabilization of the lysosomic membranes of leukocytes and to the intensification in the lungs of infiltrative and destructive processes, in comparison with the course of inflammation in the nonimmunized animals. Peculiarities of the antigens used caused some specificity in the course of pneumonia. A relationship between the changes of blood leukocytes and disturbances in the lungs in immunization is supposed.

[State of the kallikrein-kinin system in blood from the heart ventricle in the dynamics of spontaneous hypertension in the rat]. / Sostoianie kallikrein-kininovoi sistemy krovi iz zheludochkov serdtsa v dinamike razvitiia spontannoi gipertenzii u krys.

Estimations of prekallikrein and kallikrein inhibitor by means of TAME-esterase method and of the content of kininogen by biological procedure were carried out in blood plasma, isolated from right and left ventricles of heart, of rats with spontaneous hypertension, Okamoto-Aoki strain, and of the normotensive animals Wistar-Kyoto strain. The animals were 2=3 months, 6=8 months and 12=14 months old. Development of spontaneous hypertension was accompanied by an increase in the kallikrein-kinin system components studied. Content of prekallikrein and kininogen was higher in blood plasma from left heart ventricle than in blood from right ventricle, independently of the age of the rats, by 6=10% and 33=40%, respectively. Lung tissue was shown to carry out a definite reserve function in the compensatory increase of the kallikrein-kinin system potential under conditions of spontaneous hypertension.

[Effect of precipitating antibodies to pancreatic kallikrein on arginine esterase contact activation in human and rabbit blood]. / Vliianie pretsipitiruiushchikh antitel k pankreaticheskomu kallikreinu na kontaktnuiu aktivatsiiu argini-ésterazy krovi cheloveka i krolika.

Effect of precipitating antibodies against pig pancreatic kallikrein on the BAEE-esterase activity was studied in human blood plasma and in rabbit blood serum, activated by kaolin. An inhibitory effect of the antibodies on the increased BAEE-esterase activity was observed within the first minute of incubation after treatment with kaolin. The inhibition of the BAEE-esterase activity constituted 65.1% in human blood plasma and 40.9% in rabbit serum. Possible mechanism is discussed on the inhibition of contact activation of arginine esterase in human and rabbit blood by antibodies against pancreatic kallikrein.

[Contact-activated and immunoreactive prekallikrein in the plasma of patients with hypertension]. / Kontaktno-aktiviruemyi i immunoreaktivnyi prekallikrein v plazme krovi bol'nykh gipertonicheskoi bolezn'iu.

Alterations in activity of kaolin-activated (contact-activated, CA) and immunoreactive (IR) prekallikrein were studied in blood plasma of healthy persons and of patients with hypertension under conditions of rest and of dose-dependent physical loading. CA prekallikrein was dissimilarly altered depending on the type of hemodynamic structure of the pressure reaction (arterial pressure): content of CA prekallikrein was increased in hyperkinetic type and--decreased in hypokinetic type. Content of IR prekallikrein, measured by means of radial immunodiffusion using monospecific antiserum, was similar in healthy persons and in the patients at rest and after physical exercises. It was increased slightly but did not correlate with CA prekallikrein alterations. As content of CA- and IR-prekallikreins did not correlate in the disease dynamics and after physical exercises, functional impairments may cause activation of blood plasma prekallikrein in hypertension. A procedure for isolation of highly purified preparation of kallikrein, production of monospecific antiserum to prekallikrein are described. Immunochemical studies and quantitative estimation of IR-prekallikrein were carried out.

[Acid-stable proteinase inhibitor from the urine of pregnant women, the properties of the highly purified preparation and the isolation of a monospecific antiserum]. / Kislotostabil'nyi ingibitor proteinaz iz mochi beremennykh zhenshchin; svoistva vysokoochishchennogo preparata; poluchenie monospetsificheskoi antisyvorotki.

Two highly purified forms of acid-stable proteinase inhibitor from urine of pregnant women with nephropathies (Mr 22000 and 32000, ASI-22 and ASI-32, respectively) were obtained. The preparations were homogenous in molecular mass and polymorphous in molecular charge (pI from 3.9 to 4.2). ASI-22 an ASI-32 effectively inhibited trypsin and chymotrypsin (Ki approximately 1 x 10(-8)-1 x 10(-9) M, ka approximately 10(5)M-1 sec-1, kd approximately 3 x 10(-4) sec-1) by the permanent mechanism of action. Both forms inhibited the esterase activity of pancreatic pig elastase by the progressive mechanism of action (ki approximately 1 x 10(4)M-1 min-1 at 37 degrees). Rabbit monospecific antiserum to total ASI-22 and ASI-32 preparation was obtained 1 ml of the anti-ASI-serum contained 95 micrograms of antibodies. Total ASI preparations was immunochemically homogenous and had antigenic similarity to inter-alpha-trypsin inhibitor from human plasma.