Search PubMed⌕ Search

Biomedical subjects

R Gatti

Publications and source records attributed to R Gatti.

At least 199 records · Page 11Linked to original sources
PubMed1982

Mucolipidosis IV, a sialolipidosis due to ganglioside sialidase deficiency.

A female patient of Italian, non-Jewish, descent, 22 years of age, with cloudy corneae, capsular lens opacities and severe and progressive mental and motor deterioration is described. Ultrastructural examination of a skin biopsy sample showed storage of membranous cytoplasmic bodies in Schwann cells, vessel walls, fibroblasts, smooth muscle fibres and sweat glands, and the presence of some electron-lucent vacuoles, filled with fibrillo-granular material, in sweat glands. Biochemical analysis of cultured fibroblasts (from skin explant) showed a complete deficiency of the sialidase acting on gangliosides, while the sialidase acting on sialyllactose and MU-NeuAc, and several lysomal hydrolases were normal. The urine sediment analysis showed accumulation of all phospholipid species, of several glycolipids and of gangliosides, especially of the polysialylated species. We conclude that the patient under examination is affected by Mucolipidosis IV and the term 'sialolipidosis' is suggested for this inborn disorder.

Adult↗
PubMed1982

Peptide map comparisons of similar serologically defined HLA-DR antigens isolated from different lymphoblastoid cell lines.

In these studies, we have examined the possibility that DR subtypes, closely related to each other in structure, compose the major DR allotypic groups. The structures of DR molecules, isolated from pairs of cell lines that had the same serologically defined HLA-DR type, were compared by peptide mapping. HLA-DR molecules isolated from pairs of cell lines in which both members were either DR1, DR2, or DR7 were identical. However, DR molecules isolated from cell lines LG-29 (DR5) and LG-38 (DR5) displayed two distinct differences in their small (beta) subunits. This number of differences, two of 15 peptides or 15%, is much fewer than is observed between DR allotypes (approximately 50%) and suggests that at least two subtypes exist within the DR5 allotype family.

Animals↗
PubMed1982

[Synthesis of 2-methoxynaphthalene derivatives as potential anti-inflammatory agents].

Compounds having 2-methoxynaphthalene as their parent nucleus were synthesized and evaluated for antiinflammatory effect according to the carrageenin paw edema method in rats. The synthetic routes for the preparation of isomeric 1,2- and 2,6-disubstituted derivatives are described. Replacement of the alpha-methylacetic moiety in naproxen by 4-hydroxybutyric acid side chain did not cause loss of activity.

Animals↗
PubMed1981

Antiviral compounds. XVII - Synthesis and in vitro antiviral activity of 1-substituted 2-chloro- and 2-methoxyindole-3-carboxaldehyde thiosemicarbazones.

A series of indole-3-carboxaldehyde thiosemicarbazones with substituent variations at the 1 and 2 positions were prepared and evaluated for in vitro antiviral activity against vaccinia virus strain IHD and parainfluenza virus type 3, HA-I/CR-8 strain. The inhibitory activity of most compounds was restricted to the vaccinia virus, only compounds (III e) and (III f) being effective against both viruses. Structure-activity relationship are discussed.

Antiviral Agents↗
PubMed1981

Plasma and urine free L-carnitine in human diabetes mellitus.

L-carnitine is essential for the transport of long-chain fatty acids into mitochondria and their oxidation. Recently, a relationship between plasma free fatty acids (FFA) and L-carnitine metabolism has been observed. Plasma free L-carnitine (FC), FFA, triglycerides, cholesterol, blood glucose concentration and daily excretion of FC were determined in 20 diabetic patients as well as in 18 control subjects. Both in male diabetics and in male controls, plasma FC was significantly higher than in females. Mean plasma FC was found to be significantly reduced in diabetics (21 +/- 2 vs 35 +/- 2 mumol/1 in non-diabetic subjects; p less than 0.005). Daily urinary excretion of FC was clearly lower in diabetic patients than in controls (172 +/- 34 vs 403 +/- 38 mumol/24 h; p less than 0.001). The reduced plasma FC in diabetes mellitus may be due to redistribution between circulating free and esterified carnitine and to increased utilization of FC for synthesis of acylcarnitine in tissues.

Blood Glucose↗
PubMed1981

[Synthesis and in vitro antibacterial activity of N-methylnitrone and nitrovinyl derivatives of some N-substituted 2-chloroindol-3-carboxaldehydes].

N-methylnitrones and nitrovinyl derivatives from 1-substituted-2-chloroindol-3-carboxaldehydes were synthesized and evaluated for their in vitro antibacterial activity. Some nitrovinyl derivatives displayed good in vitro activity against Gram-positive bacteria; the compound (II e), 1-(o-chlorobenzyl)-2-chloro-3-(2-nitroethenyl)indole, was more active than nitrofurantoin against Staphylococcus aureus and Streptococcus pyogenes. Some structure-activity relationships are discussed.

Anti-Bacterial Agents↗
PubMed1981

[Substances with antiviral activity. XX. Thiosemicarbazones of 1-substituted 3-formyl-2-ethoxycarbonyl(carboxy)indoles].

A series of 3-formyl-2-ethoxycarbonyl(carboxy)indole thiosemicarbazones 1-substituted were synthesized and evaluated for in vitro antiviral activity against vaccinia virus strain IHD. The thiosemicarbazone of 1-(m-chlorobenzoyl)-3-formyl-2-ethoxycarbonylindole [(II g); Table II] was the most active compound. Some structure activity relationships are discussed.

Antiviral Agents↗
PubMed1980

Studies on the sialidoses: properties of human leucocyte neuraminidases.

At least two components of neuraminidase (acylneuraminyl hydrolase, EC 3.2.1.18) can be distinguished in human leucocytes on the basis of pH optimum, thermolability at 30 degrees C and the effect of the detergent octyl-beta-D-glucoside. With 4-methylumbelliferyl-alpha-D-N-acetylneuraminate as substrate, the A component has a pH optimum of 5.0, is labile at 30 degrees C and is unaffected by 0.2 M octyl-beta-glucoside. The B component has a pH optimum of 4.0-4.2, is stable at 30 degrees C but loses most of its activity in the presence of 0.2 M octyl-beta-glucoside. Both A and B components are membrane-bound but only the A component is solubilized by octyl-beta-glucoside in an active form. Molecular weights of neuraminidases by gamma-ray radiation inactivation (a method that does not require solubilization of the enzyme) were found to be 240 000 +/- 19 000 for the B component, 203 000 +/- 17 000 for the A component and 238 000 +/- 8000 for the octyl-beta-glucoside-solubilized A component. Gel filtration of soluble A component on Sephacryl S-300, in the presence of octyl-beta-glucoside, showed a single peak of activity eluted at or near the void volume suggesting that the enzyme is still in an aggregated form. Profound deficiency of neuraminidase activity was found for both A and B components in leucocytes of patients affected with sialidoses type 1 and 2 (less than 15% normal) and intermediate activity in obligate heterozygotes. These results suggest that the A and B components of leucocyte neuraminidase are closely related from the genetic point of view and that rapid diagnosis of sialidoses can be done by fluorimetric assay of neuraminidase in leucocytes.

Adolescent↗
PubMed1980

[Substances with antiviral activity. XIII. Synthesis and in vitro activity of thiosemicarbazones of 1-benzoyl- and 1-benzyl-2-chloro-3-formylindoles].

A series of 1-benzoyl- and 1-benzyl-2-chloro-3-formylindole thiosemicarbazones was synthesized and investigated for antiviral activity against vaccinia virus IHD strain and parainfluenza virus type 3, HA-I/CR-8 strain. The results confirmed the previously observed high activity against vaccinia virus of m-substituted benzoyl derivatives. On the contrary, the analogous benzyl derivatives showed marginal activity; the compound (V c), however, was the most active against the parainfluenza virus.

Antiviral Agents↗
PubMed1980

[Fine structure of pineal gland in some Chiroptera. (Vesperugo savi and Rinolophus f.e.). I. Histochemical and ultrastructural study (author's transl)].

Fine structure of pineal gland was studied in some Chiroptera and compared with those of other micromammals. Three cells types were found in pineal parenchyma: pinealocytes, the most numerous cells, glial cells and Mastzellen. Some nervous cells were found in peripheral dorsal pineal gland. Pinealocytes show a clear or dark matrix which are different functional stages of the same cell. A large nucleus with irregular profiles and deep invaginations, several mitochondria and ribosomes, canalicular rough surfaced endoplasmic reticulum, large Golgi apparatus, lipid droplets, secretory and heterogeneous granules distinguish this cellular type. Centrioles often joined with a striated root (formed by alternating clear and dark discs), cytoplasmic process extending in the intercellular space as far as 3-4 mu including cilia showing a 9+0 pattern, osmiophilic vacuoles involved in secretion processes were observed too. Glial cells irregularly arranged tend to form small clusters: they show a dense and glycogen particles rich matrix. This matrix was very positive for non specific esterases reaction. Blood vessels are provided by a continuous endothelium resting on basal lamina, surrounded by a thick perivascular layer. Collagen fibers, pinealocytes processes, non-myelinated nerve fibers and Mastzellen were found in pervascular area.

Animals↗
PubMed1979

Detection of carriers and prenatal diagnosis for fucosidosis in Calabria.

A significant proportion of patients affected with fucosidosis have Italian ancestors. We assayed for alpha-fucosidase activity purified mononuclear cells and/or leukocytes obtained from 64 members of two large pedigrees from Calabria, in which seven children had been diagnosed as affected with fucosidosis. Of these 64 individuals, 22 were diagnosed as carriers, while the values for the remainder were within normal limits, indicating a clear bimodal distribution among individuals at risk. These data confirm that carrier detection for fucosidosis requires the measurement of alpha-L-fucosidase activity in purified mononuclear cells from peripheral blood. In addition, this program has made prenatal identification of an affected fetus possible.

Amniotic Fluid↗