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Biomedical subjects

R Gatti

Publications and source records attributed to R Gatti.

At least 217 records · Page 12Linked to original sources
PubMed1979

Relationship between alpha-L-fucosidase deficiency in plasma and alpha-L-fucosidase activity in leukocytes.

After testing a population sample of 185 hospitalized Italian children for the plasma alpha-L-fucosidase deficiency and establishing an approximate threshold value between heterozygotes and wild-type homozygotes, we analyzed by two statistical methods the distribution of the two genotypes. The results obtained by probit analysis agree with threshold and average values expected on the basis of the Hardy-Weinberg equilibrium. In addition, the level of alpha-fucosidase in leukocytes of 12 individuals with deficiency of alpha-fucosidase in plasma was found to be significantly lower than that of 61 controls (P less than 0.005). These results indicate that the mutation(s) causing a deficiency of alpha-fucosidase in plasma is (are) also expressed in leukocytes.

Carbohydrate Metabolism, Inborn Errors↗
PubMed1979

Plasmapheresis and lymphoplasmapheresis in the management of rheumatoid arthritis.

We have demonstrated the efficacy of therapeutic pheresis in a number of rheumatic diseases, especially rheumatoid arthritis (RA). Ten of 12 patients with RA went into remissions averaging 4 months. These patients were pheresed 20 times over 11 weeks in a tapering fashion on a Haemonetics Model 30 Blood Processor. Clinical remissions were sustained even though serologies, immunoglobulins, immune functions, sedimentation rates, and circulating immune complexes returned to their pre-pheresis baseline by pheresis number 20. All these patients were taking gold or D-penicillamine concurrently, but neither of the 2 patients who failed to respond was on these agents. Plasmapheresis was just as effective as lymphoplasmapheresis. It is theorized that removal of a plasma factor that modulates lymphocyte or neutrophil function produces remissions in RA and that long-acting drugs (e.g., gold or penicillamine) are able to prevent its continued production and produce a sustained remission.

Adult↗
PubMed1978

Studies on the etiology of the experimental neuropathy from industrial adhesive (glues).

Chickens treated by paintbrushing with glue distillate (used in shoe industries), hydrocarbon mixture and TOCP in hexane, developed paralysis (4/5); in these, we have demonstrated a diffuse degeneration of the myelinic sheath of the peripheral and central neurites. Besides TOCP, cyclohexane (because of its higher concentration in the glue distillate and in the hydrocarbon mixture) may be indicated as responsible for occupational neuropathy.

Adhesives↗
PubMed1978

[Research on substances with antiviral activity. VIII. Benzindanoncarboxylic acids].

New derivatives (esters, amides, nitriles, amidines, thiosemicarbazones and guanylhydrazones) of 1H-benz[e]indene-2,3-dihydro-5-methyl-1-oxo-3-carboxylic acid and 1H-benz[e]indene-2,3-dihydro-7-methyl-3-oxo-1-carboxylic acid were prepared and tested for antiviral activities in vitro against vaccinia virus, HID stock and parainfluenza type 3 virus, HA-I/CR-8 stock. Four compounds (II g), (II i), (III b), (III g), showed weak activity in vitro against vaccinia virus.

Antiviral Agents↗
PubMed1978

[Substances having antiviral activity. IX. Synthesis and antiviral activity of 1-acyl-2-halo-3-formylindole thiosemicarbazones].

Some thiosemicarbazones of 1-acyl-2-chloro-3-formylindoles were synthesized and investigated for antiviral activity against vaccinia virus, HID stock and parainfluenza virus type 3 HA-I/CR-8 stock. Evidence of antiviral activity was found only against vaccinia virus, and was particular significant with the m-substituted 1-benzoyl-2-chloro-3-formylindoles. The first results of 2-substitution of chlorine by bromine in the indole skeleton, are reported.

Antiviral Agents↗
PubMed1977

[Research on substances with antiviral activity. VII. Activity and lipophilic of property N-alkyl-2-chloro-3-formylindole thiosemicarbazone].

Thiosemicarbazones of N-alkyl-2-chloroindole-3-carboxaldehydes were synthesized and investigated for antiviral activity against RNA virus (parainfluenza type 3, HA-I/CR-8 stock) and DNA virus (vaccinia virus, HID stock). The effect of lipophilicity on the activity against vaccinia virus was considered. Relative lipophilicities were measured by a reversed phase thin-layer chromatographic technique. The most active compound N-isopropyl-2-chloroindole-3-carboxaldehyde thiosemicarbazone appeared comparable for its activity to methisazone.

Antiviral Agents↗
PubMed1977

Reactivity of alloantibodies of the Merrit B-cell system with leukemic cells and lymphoblastoid cell lines.

Twenty lymphoblastoid cell lines were typed with 38 antisera defining the 13 groups of the Merrit B-cell alloantigenic system, and results were compared with those of a 20-member CLL cell panel. While overall pattern of Merrit groups for the two types of cell population was similar, some sera in a number of groups showed unexpected negative cytotoxic reactions with lymphoid cells compared to other sera of the same group. However, in a number of instances, these negatively reacting cells could still specificity absorb out tb Merrit antibodies, suggesting that lymphoblastoid lines may show a higher incidence of the CYNAP (cytotoxicity-negative-absorption-positive) phenomenon than CLL cells. Twelve of the lymphoblastoid lines were HLA-D homozygous and in addition, each cell line displayed positive reactions for no more than 2 of the 13 Merrit groups, while 4 of the 8 heterozygous lines were positive for 3 of the 13 groups. These findings indicate that HLA-D-homozygous cell lines are antigenically simpler than the heterozygous lines for the Merrit system and suggest that HLA-D-homozygosity may correlate with homozygosity for at least one of the Merrit loci.

B-Lymphocytes↗
PubMed1977

Sialidosis (mucolipidosis I).

The term "sialidosis" is suggested for the deficiency of alpha-neuraminidase activity in peripheral leukocytes and cultured fibroblasts which results in a considerable urinary excretion of sialyl-oligosaccharides. This defect was found in two siblings with a mild form of mucolipidosis I. 12 sialyl-acid rich oligosacharides have been isolated from the urine of the patients. The structure of ten of them has been determined. The studies of the patients show a remarkable variability of the clinical expression of this disease. The two siblings exhibited a progressive reduction of visual acuity, red-green blindness, a bilateral cherry red macular spot, punctate opacities of the lens, and minimal neurological symptoms. Morphologically, vacuolized lymphocytes, refringent inclusions in cultured fibroblasts, numerous cytoplasmatic inclusions containing a fine protein-like reticulum and some osmiophilic granules mainly in Kupffer's cells were found.

Adolescent↗
PubMed1976

Human alpha-fucosidase. Single residual enzymatic form in fucosidosis.

Four major forms of alpha-fucosidase (EC 3.2.1.51) activity were separated by isoelectrofocusing from sera of normal control individuals. All forms shifted towards less acidic pI values after neuraminidase treatment. In two patients affected with fucosidosis, only a single major acidic peak was observed and this was affected to a lesser degree by neuraminidase treatment. The kinetics of heat inactivation of the residual activity found in these two patients showed two decay rates while the controls showed only one rate. These data are considered in relation to the hypothesis of the existence of interconvertible thermolabile and thermostable forms of the enzyme which has been discussed in the preceeding paper. The residual alpha-fucosidase found in patients could be structurally altered so that its ability to form the thermostable higher molecular weight aggregates is impaired.

Adolescent↗