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Biomedical subjects

S N Shah

Publications and source records attributed to S N Shah.

At least 55 records · Page 3Linked to original sources
PubMed1983

Presynaptic tyrosine availability in the phenylketonuric brain: a hypothetical evaluation.

From measured effects of amino acids on synaptosomal tyrosine uptake and from published data on human CNS levels of amino acids hypothetical calculations were made to compare CNS tyrosine availability for catecholamine synthesis in the hyperphenylalaninemic and non-hyperphenylalaninemic condition. These calculations indicate an approximately two-fold reduction in the availability of tyrosine in phenylketonuria that is due solely to a reduction in CNS tyrosine alone.

Amino Acids↗
PubMed1983

Brain DNA metabolism in myelin deficient mutant jp, jpmsd and qk mice.

We studied metabolism of brain DNA in three myelin deficient mutants qk, jp and jpmsd mice. The DNA content, the in vivo incorporation of [14C]thymidine in DNA and the activity of acid DNase in tissues (cerebellum and cerebrum) from normal littermates and affected mice were compared. The results showed that neither the DNA content, the incorporation of [14C]thymidine in DNA nor the activity of acid DNase in brain were altered in qk affected mice. In jpmsd mice, however, the DNA content as well as the incorporation of thymidine in DNA were reduced in both cerebellum and cerebrum, but the activity of acid DNase was reduced in cerebrum only. In jp mice, although the DNA content was reduced in both cerebellum and cerebrum, the incorporation of thymidine in DNA and the activity of acid DNase were reduced in cerebrum only. The data suggest a) that in qk mutants DNA metabolism and hence cell (glial) proliferation is not affected; b) that in jpmsd mutants DNA synthesis, and thus the cell proliferation is reduced in cerebellum as well as in cerebrum of the affected mice and c) that in jp mutants the synthesis of DNA and the cell proliferation is reduced in cerebrum but not in cerebellum.

Animals↗
PubMed1983

Cholesterol ester content and activities of the ester metabolizing enzymes in jpmsd mouse brain.

The free and esterified cholesterol content, and the activities of cholesterol esterifying and the ester hydrolyzing enzyme in brain tissue from myelin synthesis-deficient mutant jpmsd mice and normal littermates were determined. Results showed that cholesterol ester content was high in the brain of jpmsd-affected mice and that the esterification of cholesterol in vitro, with or without added fatty acids was also high in the brain tissue of the affected mutant mice. The data suggest that the increase in cholesterol ester concentrations in the brain of jpmsd-affected mutant mice occurs as a result of increased synthesis rather than decreased hydrolysis.

Animals↗
PubMed1982

Effects of phenylalanine and its deaminated metabolites on Na+,K+-ATPase activity in synaptosomes from rat brain.

The effects of phenylalanine (PHE) and its deaminated metabolites phenylpyruvate (PHP), phenyllactate (PHL) and phenylacetate (PHA) on sodium and potassium activated adenosinetriphosphatase (Na+,K+-ATPase) in synaptosomes from rat brain were investigated. At very low concentrations (5-10 microM). PHE, PHL and PHA inhibited the activity, while PHP stimulated the activity. At intermediate concentrations (50-100 microM), all compounds had no effect, but at higher (0.5-1.0 mM) concentrations they inhibited the enzyme activity. Thus all the compounds tested showed a biphasic effect on the enzyme activity. Hydroxylamine inhibited the Na+,K+-ATPase activity when present alone; simultaneous addition of hydroxylamine and PHE, however, eliminated the inhibitory effects of each other. Reversal of mutual inhibition also occurred in the presence of hydroxylamine and very low (5-10 microM) concentrations of PHL or PHA. The inhibitory effects of PHE at aLl concentrations, and of PHL or PHA at low concentrations, were also eliminated in the presence of EGTA. The data indicate that inhibition of brain membrane Na+,K+-ATPase by PHE and by low concentrations of PHL and PHA may involve metal ions, but that the inhibition by high concentrations of these metabolites must occur by a different mechanism. Since Na+,K+-ATPase plays a central role in neuronal function, and the presence of excess PHE and its deaminated metabolites occurs in brain tissue under conditions of experimentally induced hyperphenylalaninemia and genetic phenylketonuria, the neurologic impairment in experimental and genetic PKU may in part be related to the deleterious effects of these compounds on brain ATPase.

Animals↗
PubMed1982

Cytosolic 3-hydroxy-3-methyl glutaryl coenzyme a synthase in rat brain: properties and developmental change.

The properties and developmental change in the activity of cytosolic 3-hydroxy-3-methyl glutaryl coenzyme A (HMG-CoA) synthase in brain was examined and whether or not HMG-CoA lyase is present in cytosol and mitochondria from brain was determined. Although mitochondrial fractions contained significant HMG-CoA lyase activity, the enzyme activity was not detected in brain cytosol. The synthase activity was present in both mitochondrial and cytosolic fraction. The HMG-CoA synthesis by brain cytosol was optimal at pH 8.0 and did not require Mg2+ or exogenous acetoacetyl CoA. This indicates that brain cytosol can synthesize sufficient quantity of acetoacetyl CoA from acetyl CoA to be utilized for HMG-CoA synthesis. Our results also showed that the specific activity (nmol acetyl CoA incorporated/mg protein) of HMG-CoA synthase in brain cytosol was high (between 2-11 days of postnatal age) when the cholesterol content of brain is increasing rapidly, and the activity declined slowly thereafter. This suggests that in brain, cytosolic enzyme HMG-CoA synthase plays a role in the regulation of cholesterol synthesis.

Acetyl Coenzyme A↗
PubMed1981

Evidence for two cholesterol ester hydrolases in human cerebrospinal fluid.

In the present study, the properties, such as pH optima, detergent requirement, and effects of various lipids, of cholesterol ester hydrolase in human cerebrospinal fluid (CSF) were examined, and the activity levels of the enzyme in CSF from multiple sclerosis (MS) patients and non-MS individuals were compared. Our data indicate that the enzyme in CSF exhibits two pH optima: pH 6.0 in the presence of Triton X-100 and pH 7.0 in the presence of sodium taurocholate. Both phosphatidylethanolamine (PE) and phosphatidylserine (PS) enhanced the hydrolase activity at pH 6.0. The activity at pH 7.0, on the other hand, was enhanced slightly in the presence of PE but was inhibited in the presence of PS. These data suggest the presence of two cholesterol ester hydrolases in CSF and also indicate that the activity at pH 6.0 may be due to microsomal enzyme in brain and that at pH 7.0 may be due to myelin enzyme. The hydrolase activity at pH 7.0 was significantly lower in CSF from MS patients. The activity at pH 6.0 in CSF from MS and non-MS patients, however, did not differ significantly. This indicates that the reduction in pH 7.0 hydrolase activity in CSF may be related to demyelination.

Carboxylic Ester Hydrolases↗
PubMed1980

Effects of alpha-methylphenylalanine plus phenylalanine treatment during development on myelin in rat brain.

The effects of hyperphenylalaninemia induced by treatment with alpha-methylphenylalanine (MPA) plus phenylalanine (PHE) on body and brain weight, on myelin and synaptosome formation, and on the lipids and fatty acids of myelin were studied in rats. The administraton of MPA (2.4 mumol/g body wt) plus PHE (2.6 mumol/g body wt) for 25 and 35 days beginning on the fifth postnatal day did not affect brain development. On doubling the dosage of PHE, body and brain weights and myelin yields were significantly lowered. The lipid composition of myelin from the brains of treated animals was largely unaffected; however, the concentration of sulfatides ws significantly reduced. Unsaturated fatty acid levels in myelin from hyperphenylalaninemic rat brains were reduced while long-chain fatty acids were unaffected. We conclude that as in hyperphenylalaninemia induced by other methods, MPA + PHE treatment impairs body and brain growth, reduces myelin formation, and causes inhibition of fatty acid desaturation in the brain.

Age Factors↗
PubMed1980

Premature pulmonary valve opening in a patient with absent tricuspid valve.

Echocardiography (M-mode and cross-sectional) disclosed premature opening of the pulmonary valve (very deep "a" wave) in a young man who underwent tricuspid valvulectomy without valve replacement 32 months before for refractory bacterial endocarditis. Cardiac catheterisation showed a prominent atrial wave on the pulmonary artery pressure curve. Thus, part of the right ventricular ejection appeared to occur before the onset of ventricular systole, as a result of powerful right atrial contraction.

Adult↗