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Biosynthesis of phosphatidylcholine from a phosphocholine precursor pool derived from the late endosomal/lysosomal degradation of sphingomyelin.

Previous studies suggest that the steps of the CDP- choline pathway of phosphatidylcholine synthesis are tightly linked in a so-called metabolon. Evidence has been presented that only choline that enters cells through the choline transporter, and not phosphocholine administered to cells by membrane permeabilization, is incorporated into phosphatidylcholine. Here, we show that [(14)C]phosphocholine derived from the lysosomal degradation of [(14)C]choline-labeled sphingomyelin is incorporated as such into phosphatidylcholine in human and mouse fibroblasts. Low density lipoprotein receptor-mediated endocytosis was used to specifically direct [(14)C]sphingomyelin to the lysosomal degradation pathway. Free labeled choline was not found either intracellularly or in the medium, not even when the cells were energy-depleted. Deficiency of lysosomal acid phosphatases in mouse or alkaline phosphatase in human fibroblasts did not affect the incorporation of lysosomal [(14)C]sphingomyelin-derived [(14)C]phosphocholine into phosphatidylcholine, supporting our finding that phosphocholine is not degraded to choline prior to its incorporation into phosphatidylcholine. Inhibition studies and analysis of molecular species showed that exogenous [(3)H]choline and sphingomyelin-derived [(14)C]phosphocholine are incorporated into phosphatidylcholine via a common pathway of synthesis. Our findings provide evidence that, in fibroblasts, phosphocholine derived from sphingomyelin is transported out of the lysosome and subsequently incorporated into phosphatidylcholine without prior hydrolysis of phosphocholine to choline. The findings do not support the existence of a phosphatidylcholine synthesis metabolon in fibroblasts.

Alkaline Phosphatase↗

Combined uridine and choline administration improves cognitive deficits in spontaneously hypertensive rats.

Rationale. Hypertension is considered a risk factor for the development of cognitive disorders, because of its negative effects on cerebral vasculature and blood flow. Genetically induced hypertension in rats has been associated with a range of cognitive impairments. Therefore, spontaneously hypertensive rats (SHR) can potentially be used as a model for cognitive deficits in human subjects. Consecutively, it can be determined whether certain food components can improve cognition in these rats. Objective. The present study aimed to determine whether SHR display specific deficits in attention, learning, and memory function. Additionally, effects of chronic uridine and choline administration were studied. Methods. 5-7 months old SHR were compared with normotensive Wistar-Kyoto (WKY) and Sprague-Dawley (SD) rats. (a) The operant delayed non-matching-to-position (DNMTP) test was used to study short-term memory function. (b) The five-choice serial reaction time (5-CSRT) task was used to assess selective visual attention processes. (c) Finally, the Morris water maze (MWM) acquisition was used as a measure for spatial learning and mnemonic capabilities. Results. (1) SHR exhibited significantly impaired performance in the 5-CSRT test in comparison with the two other rat strains. Both the SHR and WKY showed deficits in spatial learning when compared with the SD rats. (2) Uridine and choline supplementation normalized performance of SHR in the 5-CSRT test. (3) In addition, uridine and choline treatment improved MWM acquisition in both WKY and SHR rats. Conclusion. The present results show that the SHR have a deficiency in visual selective attention and spatial learning. Therefore, the SHR may provide an interesting model in the screening of substances with therapeutic potential for treatment of cognitive disorders. A combination of uridine and choline administration improved selective attention and spatial learning in SHR.

Animals↗

Molecular and biochemical characterization of an aminoalcoholphosphotransferase (AAPT1) from Brassica napus: effects of low temperature and abscisic acid treatments on AAPT expression in Arabidopsis plants and effects of over-expression of BnAAPT1 in transgenic Arabidopsis.

Aminoalcoholphosphotransferases (AAPT, EC 2.7.8.1 and EC 2.7.8.2) catalyze the transfer of CDP-aminoalcohols to sn-1, 2 diacylglycerol (DAG) to form phosphatidylaminoalcohols with the release of CMP. The Brassica napus L. AAPT1 gene (designated BnAAPT1) was identified from cDNA libraries of seedlings and developing seeds. Functional characterization was accomplished by heterologous expression of BnAAPT1 in a yeast strain deficient in AAPT activities. BnAAPT1 exhibited a greater preference for utilizing CDP-choline as a substrate with Vmax of 35 [14C]phosphatidylcholine nmol h(-1) mg(-1) protein and apparent Km of 32 microM while CDP-ethanolamine had a Vmax of 13 [14C]phosphatidylethanolamine nmol h(-1) mg(-1)protein and an apparent Km of 127 microM. The enzyme was activated by Mg2+, Mn2+ and phospholipid mixtures, and inhibited by Ca2+. A CDP-alcohol phosphotransferase motif, Asp99-Gly100-(X2)-Ala103-Arg104-(X8)-Gly113-(X3)-Asp117-(X3)-Asp121, was completely conserved in BnAAPT1 and its catalytic role was confirmed by scanning alanine mutagenesis. Over-expression of BnAAPT1 under the control of the double 35S promoter in transgenic Arabidopsis thaliana (L.) Heynh. plants led to elevated levels of the corresponding transcript and enzyme activity. In four of the high over-expression transgenic lines, phospholipid and fatty acid composition analyses revealed that chloroplastidic and extrachloroplastidic membranes isolated from transgenic leaves had about a 25% increase in phosphatidylcholine and in the proportions of polyunsaturated fatty acids [18:2+18:3], relative to the control. There were also consistent, but small differences observed in the proportions of 18:3 in transgenic green siliques and in 20:1 in mature transgenic seeds of these lines. Induction of Arabidopsis AAPT transcription in response to (+)-abscisic acid and low-temperature treatments, and the cold tolerance in BnAAPT1 transgenic seedlings implies that AAPT may play a role in resistance to damage at low growth temperatures.

Abscisic Acid↗

The beneficial effect of cholinesterase inhibitors on patients suffering from Parkinson's disease and dementia.

Patients suffering from Parkinson's disease (PD), often develop dementia (PDD). Their brain histology reveals Alzheimer's disease (AD) like changes and decreased cholin-acetyl transferase (ChAT) activity, in addition to typical PD changes. This cholinergic deficiency has been related to the degree of mental decline. As centrally acting cholinesterase inhibitors (ChEIs) provide cognitive and non-cognitive improvement for AD patients, the same therapeutic effect was hypothesized for PDD patients as well. The goal of this study was to assess the effect of ChEIs on both the cognitive and motor state of PDD patients. An open study was conducted. Eleven consecutive PDD patients (M/F 6/5 mean age 75 y) were found eligible for inclusion. They were treated for 26 weeks with tacrine (7 patients) and donepezil (4 patients) as add-on to their regular anti PD drugs. Cognitive assessment was performed at baseline and endpoint by Mini-Mental-State-Examination (MMSE) and Alzheimer's-Disease-Assessment-Scale (ADAS-cog). Global Deterioration Scale (GDS) was performed to evaluate active daily living (ADL). Motor evaluation was performed using Short Parkinson Evaluation Scale (SPES) at baseline and end-point. Statistical analysis used Student's paired t-test, ANOVA with repeated measures and Pearson correlation coefficient. ChEIs treated PDD patients showed improvement in their cognitive state. Mean ADAS-cog improved significantly by 3.2 points (p < 0.012). Mean MMSE and GDS improved non-significantly by 1.2 and 0.2 points respectively. There was no change in motor function as evident by mean SPES scores, 16.5 at baseline and endpoint. Five individuals actually demonstrated motor improvement under ChEIs. We conclude that ChEIs have a beneficial effect on the cognitive state of PDD patients without aggravating motor function.

Activities of Daily Living↗

Maternal Dietary fat and polyunsaturated fatty acids in the developing foetal rat brain.

Female rats were fed purified diets containing 10% safflower oil, which is high in linoleic acid, from approximately 2 weeks prior to mating until the 14th day of gestation. They were then fed purified diets containing safflower oil, soybean oil (containing linoleic and linolenic acids), or hydrogenated coconut oil (essential fatty acid deficient). On days 16, 18, and 21 of gestation, foetuses were removed by caesarean section and the brains were subjected to fatty acid analysis. By day 16 of gestation, the ethanolamine glycerophospholipids and combined serine-inositol glycerophospholipids were rich in polyunsaturated fatty acids, particularly arachidonic acid. Between days 16 and 21 of gestation, there was a marked increase in the C22-polyunsaturated acids in these glycerophospholipids, with 22:5n-6 deposited in foetuses from dams fed safflower or coconut oils and 22:6n-3 deposition occurring in the soybean oil group; the effects of essential fatty acid deficiency in this period were minimal. A similar pattern was evident in the choline glycerophospholipids but this fraction contained less of the polyunsaturated acids. The data are consistent with increased placental transfer of highly unsaturated fatty acids or increased foetal synthesis of these compounds during the last week of gestation, with the actual fatty acid pattern reflecting the dietary fat available to the dam.

Animals↗

The minimum vitamin requirements of the L and HeLa cells in tissue culture, the production of specific vitamin deficiencies, and their cure.

SEVEN VITAMINS HAVE TO DATE PROVED ESSENTIAL FOR THE SURVIVAL AND MULTIPLICATION OF A MOUSE FIBROBLAST (STRAIN L) AND A HUMAN CARCINOMA CELL (STRAIN HELA) IN TISSUE CULTURE: choline, folic acid, nicotinamide, pantothenic acid, pyridoxal, riboflavin, and thiamin. It was necessary to cultivate the cells for 5 to 15 days in a medium lacking the specific vitamin before the deficiency became apparent in the cessation of multiplication and the development of specific cytopathogenic effects. In their early stages these changes could be reversed by the addition of the missing vitamin, an in vitro, "cure" of a vitamin deficiency. The maximally effective concentrations were in the range 10(7) to 10(8) gm. per ml. The probability that additional vitamins not demonstrably essential under the conditions of the present experiments are nevertheless required for survival and growth is discussed in the text.

Animals↗

Intestinal calcium absorption: differences in transport between duodenum and ileum.

The interaction of calcium with sugar, sodium, and water absorption was studied in rats fed laboratory chow and in vitamin D-deficient and -repleted rats with the use of intestinal loops in vivo. Calcium absorption in the duodenum was enhanced by D-glucose only in the vitamin D-deficient state. In both vitamin D-deficient and -repleted ileum, calcium absorption decreased when NaCl was replaced by mannitol; however, it remained unchanged when NaCl was replaced by choline chloride or urea. Calcium absorption was enhanced by actively transported sugars and by increasing net water flow generated by differences in osmotic pressure and urea. Under all experimental conditions there was a strong association between net water and calcium absorption in the ileum. These findings suggest that calcium absorption is enhanced by solvent drag, although the role of sodium cannot be excluded entirely.

Animals↗

Lipid-mediated in vivo gene transfer replaces the loss of choline acetyltransferase activity after unilateral fimbria-fornix aspiration.

In Alzheimer's disease cholinergic neurons degenerate, resulting in loss of hippocampal acetylcholine. The fimbria-fornix aspiration is a well-known animal model mimicking hippocampal cholinergic deficiency. The aim of the present study was to use in vivo lipid-mediated gene transfer to introduce an expression vector coding for the acetylcholine synthesizing enzyme choline acetyltransferase into the hippocampus to replace the loss of enzyme activity after unilateral fimbria-fornix aspiration. Our data show that the lipid FuGene is useful to transfer DNA in vitro into 3T3 fibroblasts, C6 glioma cells, and primary astroglia and to express the respective enzyme. Lipid-mediated gene transfer in vivo resulted in a marked but transient expression of green fluorescent protein below the injection site peaking 5 days after the injection. Unilateral fimbria-fornix aspiration led to a marked reduction in the activity of choline acetyltransferase in the hippocampus, which was completely replaced 5 days after lipid-mediated gene transfer of the choline acetyltransferase vector. In conclusion, our data provide evidence that lipid-mediated gene transfer using FuGene is a useful tool to replace loss of choline acetyltranseferase activity in the hippocampus after fimbria-fornix aspiration; however, the lack of good gene transfer efficiency and the transient nature of expression limit its use for clinical applications.

3T3 Cells↗

An automated method for studying drug effects on problem solving by small laboratory animals.

The design and construction details of a method for studying problem solving by small animals is described. The form of motivation is escape from water in a T-shaped apparatus, thus eliminating possible confounding effects involved when food or water intake is restricted below normal. The arm of the T in which escape is possible is alternated from trial to trial. Two levels of problem solving are evaluated: an animal can minimize escape time during a series of trials by alternating its responses, i.e., 100% reinforcement; alternatively, it may choose the same alley on each trial, which results in only 50% reinforcement. The time to make the decision and the total time are automatically recorded. We have demonstrated the sensitivity of the method to hypofunctioning of the cholinergic neurotransmitter system, an analogy to behavioral changes accompanying progressive degenerative dementia. Two effects of the hypocholinergic state were especially notable: animals in this state have significantly greater difficulty in shifting from the "primitive" position habit to the more complex alternation solution, and preliminary experiments suggest that certain pharmacological treatments may be effective in overcoming this deficiency.

Animals↗

Methionine requirement of pigs between 5 and 20 kilograms body weight.

Four nursery experiments were conducted using a methionine (Met)-deficient feather meal-corn-soybean meal-dried whey basal diet (20% CP; 3,250 kcal of ME/kg, .11% choline, .19% Met, 1.00% cystine) supplemented with lysine, tryptophan, and histidine to determine the Met requirement of 5- to 10- and 10- to 20-kg pigs. Based on a true Met digestibility value of 81.6% estimated by a pig ileal digestibility assay, the Met-deficient basal diet contained .155% of digestible Met. A preliminary experiment (Exp. 1) indicated that pigs fed the Met-deficient basal diet when fortified adequately with Met could produce weight gains similar to those of pigs fed a 20% CP practical corn-soybean meal-dried whey diet. In Exp. 2 and 3, crossbred pigs weighing 5.8 kg initially were fed diets containing graded levels of digestible Met between .195 and .355%. Average daily gain increased quadratically (P less than .05) as the level of Met increased. When the data of Exp. 2 and 3 were examined together, the digestible Met requirement of 5- to 10-kg pigs was estimated to be .255% of the diet. In Exp. 4 and 5, crossbred pigs averaging 10 kg were fed digestible Met concentrations ranging from .155 to .315%. Average daily gain increased quadratically (P less than .05). The digestible Met requirement of 10- to 20-kg pigs was estimated at .255% for maximal weight gain, which was similar to that of 5- to 10-kg pigs. Assuming an 89% digestibility of Met in practical corn-soybean meal diets, the total Met level needed in practice would be .29%.(ABSTRACT TRUNCATED AT 250 WORDS)

Amino Acids, Sulfur↗

Cobalt-vitamin B12 deficiency and the activity of methylmalonyl CoA mutase and methionine synthase in cattle.

Cobalt deficiency was induced in cattle by feeding two groups of animals either a basal diet that was very low in Co (12.9-17.6 micrograms Co per kg), or the same diet supplemented with cobalt, for a total of 64 weeks. Vitamin B12 deficiency was induced, as judged by hepatic concentrations of vitamin B12 and plasma concentrations of MMA. However, the activity of holo-methylmalonyl CoA mutase was significantly reduced only in brain. This was reflected in very minor alterations in the tissue concentrations of branched chain- and odd numbered-fatty acids. The activity of holo-methionine synthase was significantly reduced in liver and brain, but there were no consequent alterations in the concentrations of phosphatidyl choline and phosphatidyl ethanolamine. This study confirms that cattle are less susceptible to the effects of cobalt deficiency than sheep, and concludes that prolonged cobalt deficiency had little significant effect on tissue metabolism.

5-Methyltetrahydrofolate-Homocysteine S-Methyltran↗

Isolation of a somatic-cell mutant defective in phosphatidylserine biosynthesis.

Mutant clones of Chinese hamster ovary (CHO) cells defective in the base-exchange reaction of phospholipids with choline were isolated by using an in situ enzymatic assay for the reaction in cell colonies immobilized on polyester cloth. The specific activities of the choline-exchange reaction in extracts of one of the mutants (designated 64) grown at 33 degrees C and 40 degrees C were 13% and 6% of those in parental (CHO-K1) cells, respectively. The choline-exchange activity in the mutant was more thermolabile in cell extracts than that in the parent, suggesting that a mutation in the structural gene for the choline-exchange enzyme might have been induced in this mutant. In culture medium supplemented with lipoprotein-deficient serum, mutant 64 grew almost normally at 33 degrees C but divided only twice at 40 degrees C and then stopped growing. Labeling of intact cells with [32P]Pi showed that mutant 64 was also strikingly defective in the biosynthesis of phosphatidylserine at 40 degrees C but was normal at 33 degrees C. Most temperature-resistant revertants of mutant 64 exhibited nearly normal ability to synthesize phosphatidylserine at 40 degrees C and also showed choline-exchange activity similar to that in parental cells. The addition of phosphatidylserine to medium supplemented with newborn calf serum, in which mutant 64 grew more slowly than parental cells at 40 degrees C, restored the growth rate of the mutant to the parental level. Our findings suggest that the choline-exchange enzyme functions as the major route for the formation of phosphatidylserine and that the temperature-sensitive growth of mutant 64 is due to a defect in phosphatidylserine biosynthesis at 40 degrees C.

Animals↗

DNA-based prenatal diagnosis for severe and variant forms of multiple acyl-CoA dehydrogenation deficiency.

OBJECTIVES: Multiple acyl-CoA dehydrogenation deficiency (MADD) is a clinically heterogeneous disorder of mitochondrial fatty acid, amino acid, and choline oxidation due to mutations in the genes encoding electron transfer flavoprotein (ETF) or ETF ubiquinone oxidoreductase (ETFQO). So far, prenatal diagnosis of MADD has relied mostly on second-trimester biochemical analyses of amniotic fluid or cultured amniocytes. We report here on an alternative DNA-based approach for prenatal diagnosis in pregnancies at risk of MADD. METHODS: We used our knowledge of the mutational status in three unrelated families with a history of MADD to perform direct sequencing for the familial mutations using genomic DNA isolated from chorionic villus samples (CVS) at gestational week 10 to 11. RESULTS: Within two days, we were able to carry out accurate DNA-based prenatal testing in one pregnancy at risk of severe MADD, and in two pregnancies at risk of variant forms of MADD. CONCLUSION: This is the first report of DNA-based prenatal diagnosis of MADD. Our molecular approach is suitable for fast and reliable first-trimester prenatal diagnosis in pregnancies at risk of severe and variant forms of MADD.

Acyl-CoA Dehydrogenase↗

Contribution of the MTHFR gene to the causal pathway for depression, anxiety and cognitive impairment in later life.

Homocysteine (Hcy) is harmful to neurons and blood vessels, including the cerebral microvasculature. It is possible that such effects contribute to the cascade of events that leads to cognitive decline, dementia, and depression in later life. Hcy is produced during the metabolism of the essential amino-acid methionine, which also involves a methyl group transfer derived from folate and choline metabolism. Its plasma level can be influenced by factors such as age, vitamin deficiency, renal function, and a common mutation in the methylenetetrahydrofolate reductase (MTHFR) gene, where cytosine is replaced by thymidine (C-->T) at nucleotide position 677. Subjects with the TT genotype have higher homocysteine levels and may be particularly prone to experiencing depression as a result of high plasma Hcy and dysfunction of methylation metabolic pathways critical to the synthesis of noradrenaline and serotonin. We designed the present study to investigate whether older women with the TT genotype would have higher depression and lower cognitive scores than women with CT and CC genotypes. A total of 240 community-dwelling women aged 70 years or over volunteered to take part in the study - 29 carried the TT genotype, 113 the CT and 98 the CC genotype. The Beck Depression Inventory (BDI) score for subjects with the TT genotype was statistically similar to the other groups (P = 0.609). Plasma Hcy showed a modest and significant correlation with BDI scores (r = 0.21) that was independent from age, B12 and folate levels. There was no association between beck anxiety inventory (BAI) scores and MTHFR genotype or homocysteine levels. The cognitive assessment of participants included measures of verbal memory, memory for faces, verbal fluency, visuo-spatial abilities and the cognitive section of the Cambridge Examination For Mental Disorders Of The Elderly (CAMCOG)-MTHFR genotype had no clear association with cognitive scores. These results indicate that, in isolation, the MTHFR C677T gene variation does not play an important role in the modulation of mood and cognitive performance in later life.

Aged↗

Cholinergic mechanisms in startle and prepulse inhibition: effects of the false cholinergic precursor N-aminodeanol.

We examined the effects of cholinergic deficiency on prepulse inhibition (PPI) of the acoustic startle. Rats treated with a choline-free diet that contained the false cholinergic precursor N-aminodeanol showed great deficit in PPI. This deficit does not appear to be secondary to an increase of stereotyped behaviors. Startle threshold was also greatly reduced, as these rats startled to the 70-dB prepulse and the baseline startle amplitude was increased by 60% over the control rats. Arecoline (4 mg/kg) partially reversed the deficit in PPI. This improvement persisted beyond the period of drug treatment. On the other hand, scopolamine (1 mg/kg) reduced PPI in the control rats. These results suggest that cholinergic systems play a major role in both the elicitation and prepulse inhibition of startle.

Acoustic Stimulation↗

Enhanced acetylcholine release in the hippocampus of cannabinoid CB(1) receptor-deficient mice.

We examined whether acetylcholine release in the hippocampus and striatum and noradrenaline release in the hippocampus is altered in CB(1) receptor-deficient mice. The electrically evoked tritium overflow from hippocampal slices preincubated with [(3)H]-choline was increased by about 100% in CB(1)(-/-) compared to CB(1)(+/+) mice whereas the electrically evoked tritium overflow from striatal slices preincubated with [(3)H]-choline and from hippocampal slices preincubated with [(3)H]-noradrenaline did not differ. The cannabinoid receptor agonist, WIN 55,212-2, inhibited, and the CB(1) receptor antagonist, SR 141716, facilitated, the evoked tritium overflow from hippocampal slices (preincubated with [(3)H]-choline) from CB(1)(+/+) as opposed to CB(1)(-/-) mice. Both drugs did not affect the evoked tritium overflow from striatal slices (preincubated with [(3)H]-choline) and from hippocampal slices (preincubated with [(3)H]-noradrenaline) from CB(1)(+/+) and CB(1)(-/-) mice. The selective increase in acetylcholine release in CB(1)(-/-) mice may indicate that the presynaptic CB(1) receptors on the cholinergic neurones of the mouse hippocampus are tonically activated and/or constitutively active in vivo.

Acetylcholine↗

Protein kinase A regulates cholinergic gene expression in PC12 cells: REST4 silences the silencing activity of neuron-restrictive silencer factor/REST.

The role of protein kinase A in regulating transcription of the cholinergic gene locus, which contains both the vesicular acetylcholine transporter gene and the choline acetyltransferase gene, was investigated in PC12 cells and a protein kinase A-deficient PC12 mutant, A126.1B2, in which transcription of the gene is reduced. The site of action of protein kinase A was localized to a neuron-restrictive silencer element/repressor element 1 (NRSE/RE-1) sequence within the cholinergic gene. Neuron-restrictive silencer factor (NRSF)/RE-1-silencing transcription factor (REST), the transcription factor which binds to NRSE/RE-1, was expressed at similar levels in both PC12 and A126.1B2 cells. Although nuclear extracts containing NRSF/REST from A126.1B2 exhibited binding to NRSE/RE-1, nuclear extracts from PC12 cells did not. The NRSF/REST isoform REST4 was expressed in PC12 cells but not in A126.1B2. REST4 inhibited binding of NRSF/REST to NRSE/RE-1 as determined by gel mobility shift assays. Coimmunoprecipitation was used to demonstrate interaction between NRSF/REST and REST4. Expression of recombinant REST4 in A126.1B2 was sufficient to transcriptionally activate the cholinergic gene locus. Thus, in PC12 cells, protein kinase A promotes the production of REST4, which inhibits repression of the cholinergic gene locus by NRSF/REST.

Acetylcholinesterase↗