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PubMed1993

Magnetic resonance imaging in juvenile Canavan disease.

We present a 2-year-old boy and a 6-year-old girl with mild Canavan disease (CD). Aspartoacylase activity in skin fibroblasts was deficient. Magnetic resonance imaging (MRI) of the brain did not show the prominent leucodystrophy previously reported in CD, but there was a hyperintense signal from the lentiform nuclei and the heads of the caudate nuclei on the T2-weighted MR images. This suggests a specific vulnerability of the corpus striatum in these patients. In the older patient, the white matter became affected at the age of 6 years. Proton magnetic resonance spectroscopy (1H-MRS) of white matter revealed a normal concentration of N-acetyl-L-aspartate (NAA) and a markedly decreased concentration of choline containing compounds (Cho) in the boy but a normal ratio of NAA to Cho in the girl. We conclude that deficient NAA catabolism affects myelin metabolism. This may present as changes in the striatum and/or as a low concentration of Cho before leucodystrophy appears on MRI.

Amidohydrolases↗
PubMed1997

Dietary protein deficiency induced changes in protein kinase C activity and phospholipid metabolism in rat hepatocytes.

Dietary protein deficiency is known to alter the protein kinase C activity in various tissues of rats. Protein kinase C activity is influenced by the metabolism of membrane phosphoinositides and phosphatidyl choline (PC). For metabolic studies, hepatocytes have been the cells of choice of various workers. Therefore, studies on protein kinase C and these phospholipids were conducted in hepatocytes of rats maintained on three different diets viz. casein (20% protein) deficient (4% protein, rice flour as source of protein) and supplemented (deficient diet supplemented with L-lysine and DL-threonine) diet for 28 days. The protein deficiency in diet led to a decline in protein kinase C activity (P < 0.01) without effecting its translocation, an increase in phosphatidyl inositol 4,5-bisphosphate (P < 0.001) and a decrease in phosphatidyl inositol 4-monophosphate and phosphatidyl inositol (P < 0.01) but did not alter the PC contents, as compared to the casein group. Supplementation of deficient diet with L-lysine and DL-threonine could considerably reverse the effect of deficiency of protein in diet. The results suggest that quality of dietary protein is mainly relevant for maintaining phospholipid metabolism and physiology of hepatocytes and thus the signalling mechanism in these cells.

Animals↗
PubMed1990

The cholinergic system and memory: amelioration of ethanol-induced memory deficiency by physostigmine in rat.

Male Sprague-Dawley rats were treated with a solution of ethanol (20% v/v) as the only source of fluid for 28 weeks while controls received tap water. Profound reductions of acetylcholine content, the activities of choline acetyltransferase and acetylcholinesterase and choline uptake were observed in the cortex, hippocampus, substantia innominata and striatum four weeks after withdrawal from ethanol. These changes in cholinergic markers were accompanied by an impaired performance in a spontaneous alternation task tested at long acquisition-retention intervals. This ethanol induced behavioural deficiency, which most likely represents a memory impairment, could be reversed by application of physostigmine (0.45 g/kg; i.p) whereas neostigmine did not show any behavioural effect. The results suggest that ethanol-induced memory impairment can be ameliorated by pharmacological manipulation of central cholinergic function.

Acetylcholine↗
PubMed1982

Lipid peroxides in brain during aging and vitamin E deficiency: possible relations to changes in neurotransmitter indices.

Lipid peroxide levels, were found to be significantly higher in brains of 18 month old as compared to 4 month old rats, with particularly large increases occurring in the olfactory bulb, globus pallidus, cerebral cortex and caudate-putamen (CP). Eighteen month old rats fed a vitamin E deficient diet for 9 months before sacrifice had lipid peroxide levels significantly higher than age-matched controls in the cerebral cortex, hippocampus and hypothalamus. Age-related decreases were seen in choline acetyltransferase, acetylcholinesterase and 3H-QNB binding in some but not all brain regions, while GABA transaminase and MAO showed age-related increases. No age-related change was seen in tyrosine hydroxylase in the CP or in 3H-dihydroalprenolol (DHA) or 3H-spiroperidol binding in the cortex. As compared with controls, vitamin E deficient rats showed decreases of 38% in cortical 3H-DHA binding, of 33% in 3H-QNB binding in the CP and of 23% and 12% in choline acetyltransferase in the CP and cerebellum, respectively. There were no completely consistent regional correlations between significant changes in lipid peroxidase levels and any neurotransmitter indices studied except for MAO which was only measured in the caudate-putamen.

Aging↗
PubMed2006

Brucella abortus synthesizes phosphatidylcholine from choline provided by the host.

The Brucella cell envelope is characterized by the presence of phosphatidylcholine (PC), a common phospholipid in eukaryotes that is rare in prokaryotes. Studies on the composition of Brucella abortus 2308 phospholipids revealed that the synthesis of PC depends on the presence of choline in the culture medium, suggesting that the methylation biosynthetic pathway is not functional. Phospholipid composition of pmtA and pcs mutants indicated that in Brucella, PC synthesis occurs exclusively via the phosphatidylcholine synthase pathway. Transformation of Escherichia coli with an expression vector containing the B. abortus pcs homologue was sufficient for PC synthesis upon induction with IPTG (isopropyl-beta-d-thiogalactopyranoside), while no PC formation was detected when bacteria were transformed with a vector containing pmtA. These findings imply that Brucella depends on choline provided by the host cell to form PC. We could not detect any obvious associated phenotype in the PC-deficient strain under vegetative or intracellular growth conditions in macrophages. However, the pcs mutant strain displays a reproducible virulence defect in mice, which suggests that PC is necessary to sustain a chronic infection process.

Amino Acid Sequence↗
PubMed1977

Fatty acid composition of myelin isolated from the brain of a patient with cellular deficiency of co-enzyme forms of vitamin B12.

Myelin fractionation and subsequent lipid isolation have been carried out on a brain from a patient who suffered from a cellular deficiency of the adenosylcobalamin and methylcobalamin co-enzyme forms of vitamin B12. Examination of the fatty acid composition of choline and ethanolamine glycerophospholipids indicated a relative enrichment of odd-chain fatty acids which were identified by gas-liquid chromatography-mass spectroscopy as C15, C15:1, C17 and C17:1. A mixture of methyl branched C17 fatty acids was also identified. Odd-chain fatty acids accounted for 9.8% of the total fatty acid in the myelin choline phospholipid conpared to control values of 1.2%. The affected brain myelin phospholipids had a lower unsaturated fatty acid content. Examination of the myelin sphingolipids, sphingomyelin, cerebroside and sulfatide, yielded abnormal fatty acid profiles. The sphingomyelin contained only small amounts of C24:1 fatty acid. Both normal and hydroxy fatty acid containing cerebroside and sulfatide had reduced levels of C24 fatty acid. Determination of the relative hydroxy and normal fatty acid content of the galactolipids indicated an abnormally high hydroxy fatty acid level. Abnormal fatty acid profiles of brain cerebral sphingolipids have not been previously described in cases of vitamin B12 deficiency. Whether or not these alterations are characteristic will only be established by estimating sphingolipids in other such cases.

Brain Chemistry↗
PubMed2000

Cholinsalicylate gel induced oral lesion: report of case.

Salicylic acid and its derivatives are extensively used medications for the treatment of systemic and local diseases. However, injudicious use of aspirin as well as other derivatives of salicylic acid, may cause systemic and oral complications such as mucosal burns and oral ulcers. In children, topical administration of these drugs, even in small dosages, may cause adverse reactions. This report shows a case of an 8 year old boy with G6PD deficiency, who had a mucosal burn caused by application of a cholinsalicylate paste. Three days later, the child developed oral ulcers, malaise and fever. The present case is characteristic of the enigmatic nature of the etiology and diagnosis of oral lesions, and the possible connection between cholinsalicylate systemic absorption and hemolytic anemia is discussed.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMed1975

Active and passive Ca2+ fluxes across cell membranes of the guinea-pig taenia coli.

The exchange of Ca between the extracellular fluid and the cellular compartment has been investigated in smooth muscle cells of taenia coli. It was found that during the initial phase of metabolic depletion by DNP + IAA, the net inwards flux of Ca amounts to 0.02 pmol cm(-2)-sec(-1). This increase might be proportional to the physiological calcium leak. The study of the relation between the inwardly directed Na gradient and the cellular Ca content has revealed that this Na gradient exerts no effect during prolonged exposure to K-free solution and a very limited effect during exposure to Na-deficient solutions. The cellular 45Ca release induced by metabolic inhibition is not affected by substituting Li or choline for Na. The supplementary calcium which enters the cells during exposure to a solution at low temperature is extruded on returning to a solution at 35 degrees C, even if the Na gradient is reversed. This finding and the effects of metabolic inhibition indicate that Ca extrusion in smooth muscle cells is a process which depends on metabolism and which is not affected by the inwardly directed Na gradient.

Adenosine Triphosphate↗
PubMed1979

Friedreich's ataxia 1979: an overview.

This overview summarizes the investigations carried out during the second part of Phase Two of the Quebec Cooperative Study of Friedreich's Ataxia. These investigations outline in more details the fundamental role played by an abnormality in the fatty acid composition (deficient linoleic acid, 18:2) of the cholesterol esters of high density lipoproteins (HDL) in the phenotypic expression of the disease. They postulate a defective incorporation of linoleic acid to surface phosphatidylcholine of chylomicrons and consequent relative and absolute decreases in lipoprotein protein components because of overpacking with defective cholesteryl esters. Secondarily to these changes, the postulated lack of activation of the lipoamide dehydrogenase (LAD) of the pyruvate dehydrogenase (PDH) complex could result in slow pyruvate oxidation, glucose intolerance, deficient synthesis of acetylcholine, and depletion of glutamic and aspartic acid pools. In parallel, abnormal phosphatidyl-choline molecules could be incorporated to membranes, resulting in specific defects in some functions of these membranes, including transport of calcium and/or taurine and myelinization. The framework of an understanding of Friedreich's ataxia is now available, but much fundamental and clinical work remains to be done to fill in and prove each one of these postulated steps.

Friedreich Ataxia↗
PubMedDate not supplied

Cranial MR spectroscopy of tetrahydrobiopterin deficiency.

BACKGROUND AND PURPOSE: Severe and progressive neurologic disease remains a problem for patients with hyperphenylalaninemia due to a deficiency of tetrahydrobiopterin (BH4), even with early diagnosis and despite treatment with BH4 and neurotransmitter precursors. Few reports have included the associated imaging characteristics. Our purpose was to describe the imaging features of BH4-deficient patients identified by neonatal screening in a Taiwanese population and to correlate the imaging features with the treatment. METHODS: This study analyzed the cases of eight BH4-deficient patients who were examined by MR imaging and MR spectroscopy. Analysis of the findings was correlated with the clinical findings. RESULTS: One patient whose intelligence quotient score was lower than those of the other seven patients experienced seizures in conjunction with central white matter signal changes on MR images and a lactate peak on MR spectroscopy. Lactate peak was revealed in another patient who had marked elevations of N-acetylaspartate:creatine and N-acetylaspartate:choline ratios. Although most patients had a higher than average N-acetylaspartate:creatine or N-acetylaspartate:choline ratio, the patient who had decreases of both ratios possessed the highest intelligence quotient scores among the eight patients. In addition, the myoinositol:choline ratio correlated positively with the average BH4 dosage (P =.027, r = 0.027) and the choline:creatine ratio correlated negatively with the average 5-hydroxytryptophan dosage (P =.035, r = -0.742). CONCLUSION: Compared with classical phenylketonuria, patients with BH4 deficiency have fewer white matter changes revealed by MR imaging but more changes revealed by MR spectroscopy. MR spectroscopy is a potential method with which to monitor the dosages of supplements used to treat this disorder. In addition, MR spectroscopy may be helpful in gaining understanding of the neurophysiological changes that occur in association with this disease.

Antioxidants↗
PubMed1995

Targeted inactivation of the mouse alpha 2-macroglobulin gene.

The mouse alpha 2-macroglobulin gene was inactivated in embryonic stem cells by homologous recombination. Liver alpha 2-macroglobulin mRNA and plasma protein was absent in homozygotes and reduced to 50% in heterozygotes. alpha 2-Macroglobulin-deficient mice were viable and produced normally sized litters with normal sex ratio over 3 generations. Characterization of adult homozygotes included diets with different fat content, treatments with endotoxin, bleomycin, carbon tetrachloride, and ethionine to test for immune system, lung, liver, and pancreas toxicity, respectively. Knock-out mice were more resistant to endotoxin but more sensitive to a choline-free diet supplemented with ethionine. Regulation of murinoglobulin mRNA expression during pregnancy was analyzed as a possible back-up mechanism for the deficiency in alpha 2-macroglobulin. In addition, expression of mRNA was studied, coding for alpha 2-macroglobulin receptor/lipoprotein receptor-related protein, low density lipoprotein receptor, and very low density lipoprotein receptor and for some common ligands, i.e. apolipoprotein E, lipoprotein lipase, and the 44-kDa heparin binding protein. Their differential regulation in the knock-out mice relative to C57B1 mice was evident and is discussed. The impressive 15-fold increase in maternal liver murinoglobulin mRNA at partum in the knock-out mice indicated increased consumption, compared to only 4-fold in normal mice. Thus, murinoglobulin appears as the major proteinase inhibitor around partum, obviously solicited to a much greater extend in alpha 2-macroglobulin-deficient mice.

Animals↗
PubMed1978

Fatty acid composition of submandibular salivary gland lipids in essential fatty acid deficient rats.

Essential fatty acid (EFA) deficiency was induced in young weanling rats by feeding a fat-free diet or a diet containing 7% Hydrogenated coconut oil (HCO). At intervals of 4, 8, and 16 weeks, rats from the deficient and the control groups were killed and their Submandibular Salivary Glands (SMSG) were dissected out and extracted for lipids. The fatty acid composition of total lipids, triglycerides (TG), free fatty acids (FFA), phosphatidyl choline (PC), and phosphatidyl ethanola-mine (PE) fractions was determined. The levels of 16:1 and 18:1 were increased whereas those of 18:2 and 20:4 were decreased in the EFA-deficient groups as compared to the controls. 5,8,11-eicosatrienoic acid (20:3 omega9) accumulated in the total lipids as well as other lipid fractions of the SMSG from the deficient rats. The levels of this fatty acid increased as the EFA-deficiency progressed. Changes in fatty acid composition of SMSG lipids of EFA-deficient rats were associated with a reduction in flow rate of whole saliva. Protein concentration and amylase activity of saliva was the same in the two groups.

Animals↗
PubMedDate not supplied

Biochemical and cognitive studies of apolipoprotein-E-deficient mice.

Apolipoprotein-E-deficient mice provide a useful model system for studying the role of apolipoprotein E (apoE) in brain function. In the present study, we characterized the cholinergic function of these mice and the extent of phosphorylation of their cytoskeletal protein tau. Morris water maze tasks revealed deficits in working memory that were accompanied by a specific decrease in hippocampal and cortical choline acetyltransferase activities. Immunoblot experiments utilizing native and dephosphorylated tau and antibodies directed against specific phosphorylated and unphosphorylated tau epitopes revealed that tau of the apoE-deficient mice is hyperphosphorylated. These results show that apoE-deficient mice have cognitive cholinergic and cytoskeletal derangements and point out the importance of this model for studying the role of apoE in neuronal function.

Animals↗
PubMed1984

Lung surfactant and fatty acid composition of lung tissue and lavage of rats fed diets containing different lipids.

Two nutritional models, essential fatty acid (EFA) deficiency and the feeding of saturated vs unsaturated fats, were used to determine the effects of dietary lipids on the fatty acid composition of rat lung and lavage. Semipurified diets containing 7% corn oil, 7% hydrogenated coconut oil (EFA-deficient), 10% butter or 10% safflower oil were fed to dams during lactation and thereafter to their offspring for a total of 24 weeks. Lipids were extracted from the lung lavage and lung tissue and their fatty acid composition was determined. The content of dipalmitoylphosphatidylcholine (DPPC), the main surfactant in the lungs, was also determined. The results show that the levels of DPPC in the lungs of rats fed 10% butter decreased although the decrease in the EFA-deficient rats was greater. Comparing rats fed butter with those fed corn oil, there were also modifications in the fatty acid composition of the total lipids and phospholipids of lung tissue and lavage as well as in phosphatidyl-choline, phosphatidylethanolamine and phosphatidylinositol + phosphatidylserine fractions isolated from the lung tissue. The changes in fatty acid composition were somewhat fewer in rats fed butter then in those fed an EFA-deficient diet. The results suggest that a marginal EFA deficiency produced in rats by long-term feeding of 10% butter may account for the reduction in DPPC levels and in the changes in fatty acid composition in the lung tissue and lavage.

Animals↗
PubMed1985

Red cell choline in Korsakoff psychosis.

Red blood cell (RBC) choline was measured in 48 male patients with Korsakoff psychosis secondary to alcoholism and in 19 age-matched male controls using gas chromatography-mass spectrometry. The variance in RBC choline was significantly different between Korsakoff patients and controls (p less than 0.002). RBC choline was significantly higher in Korsakoff patients compared with controls (p less than 0.02). There was no significant correlation between age and RBC choline. Higher RBC choline levels suggest differences in membrane transport properties. Differences in membrane properties may indicate a neuronal membrane vulnerability to the toxic effect of alcohol or to thiamine deficiency which lead to the degenerative changes associated with Korsakoff psychosis.

Aged↗
PubMed1977

Huntington's disease: clinical and chemical effects of choline administration.

Choline (Ch) administration sequentially increases blood Ch, brain Ch, and brain acetylcholine (ACh) levels in the rat and might be useful in Huntington's disease, a brain disorder thought to be associated with deficient cholinergic tone. Ten patients with Huntington's disease took Ch orally (8 to 20 gm per day) after the extent of their disabilities had been documented by clinical examination, movies, and combined electromyogram-accelerometer recordings. Choline levels in blood and cerebrospinal fluid increased markedly during treatment with Ch, affirming that oral Ch administration increases the amount of ACh precursor delivered to the brain. Although some of the patients exhibited transient improvement in speech, balance, and gait, treatment with Ch failed to bring about consistent or lasting improvement in any of the subjects.

Acetylcholine↗