Abstract：
:In the present work we investigated the in vitro effect of the branched-chain amino acids (BCAA) accumulating in maple syrup urine disease (MSUD) on some parameters of energy metabolism in cerebral cortex of rats. 14CO2 production from [1-14C]acetate, [1-5-14C]citrate and [U-14C]glucose, as well as glucose uptake by the brain were evaluated by incubating cortical prisms from 30-day-old rats in the absence (controls) or presence of leucine (Leu), valine (Val) or isoleucine (Ile). All amino acids significantly reduced 14CO2 production by around 20-55%, in contrast to glucose utilization, which was significantly increased by up to 90%. Furthermore, Leu significantly inhibited the activity of the respiratory chain complex IV, whereas Val and Ile markedly inhibited complexes II-III, III and IV by up to 40%. We also observed that trolox (alpha-tocopherol) and creatine totally prevented the inhibitory effects provoked by the BCAA on the respiratory chain complex activities, suggesting that free radicals were involved in these effects. The results indicate that the major metabolites accumulating in MSUD disturb brain aerobic metabolism by compromising the citric acid cycle and the electron flow through the respiratory chain. We presume that these findings may be of relevance to the understanding of the pathophysiology of the neurological dysfunction of MSUD patients.
journal_name
Neurochem Resjournal_title
Neurochemical researchauthors
Ribeiro CA,Sgaravatti AM,Rosa RB,Schuck PF,Grando V,Schmidt AL,Ferreira GC,Perry ML,Dutra-Filho CS,Wajner Mdoi
10.1007/s11064-007-9423-9subject
Has Abstractpub_date
2008-01-01 00:00:00pages
114-24issue
1eissn
0364-3190issn
1573-6903journal_volume
33pub_type
杂志文章abstract：:The phosphoinositide signal transduction system, and particularly, phospholipase Cbeta isozymes, are relevant in the etiopathogeny of human neuropsychiatric pathologies such as depression. Stimulation of phospholipase Cbeta activity by muscarinic receptors and G proteins was determined in crude and synaptosomal membra...
journal_title：Neurochemical research
pub_type： 杂志文章
doi：10.1023/b:nere.0000026412.66508.14
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abstract：:The vesicular hypothesis has stimulated fruitful investigations on many secreting systems. In the case of rapid synaptic transmission, however, the hypothesis has been found difficult to reconcile with a number of well established observations. Brief impulses of transmitter molecules (quanta) are emitted from nerve te...
journal_title：Neurochemical research
pub_type： 杂志文章,评审
doi：10.1023/a:1022451224748
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abstract：:A panel of mouse monoclonal antibodies to rat and human myelin-associated glycoprotein (MAG) was developed. Normal mice were unresponsive to rat MAG, and successful immunization with rat MAG was only achieved in autoimmune NZB mice. By contrast, all strains of mice were responsive to human MAG. The monoclonal antibodi...
journal_title：Neurochemical research
pub_type： 杂志文章
doi：10.1007/BF00964654
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journal_title：Neurochemical research
pub_type： 杂志文章
doi：10.1007/BF00965906
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journal_title：Neurochemical research
pub_type： 杂志文章
doi：10.1007/BF00971589
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journal_title：Neurochemical research
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journal_title：Neurochemical research
pub_type： 杂志文章
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journal_title：Neurochemical research
pub_type： 杂志文章
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更新日期：1976-04-01 00:00:00
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journal_title：Neurochemical research
pub_type： 杂志文章
doi：10.1007/BF00967242
更新日期：1993-04-01 00:00:00
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更新日期：2015-11-01 00:00:00
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journal_title：Neurochemical research
pub_type： 杂志文章
doi：10.1007/s11064-009-0046-1
更新日期：2010-02-01 00:00:00
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doi：10.1007/s11064-007-9288-y
更新日期：2007-07-01 00:00:00
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journal_title：Neurochemical research
pub_type： 杂志文章
doi：10.1007/BF00965016
更新日期：1986-03-01 00:00:00
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更新日期：2019-05-01 00:00:00
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journal_title：Neurochemical research
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更新日期：2007-08-01 00:00:00
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pub_type： 杂志文章
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更新日期：2002-04-01 00:00:00
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pub_type： 杂志文章,评审
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更新日期：1995-05-01 00:00:00
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journal_title：Neurochemical research
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更新日期：1986-08-01 00:00:00
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更新日期：2012-12-01 00:00:00
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journal_title：Neurochemical research
pub_type： 杂志文章
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更新日期：1977-02-01 00:00:00
abstract：:The 7S NGF complex from the male mouse submaxillary gland consists of the alpha, gamma, and beta subunits in the ratio alpha 2 gamma 2 beta. The beta (NGF) subunit contains all the known biolocial activity of 7S NGF. The alpha and gamma subunits are both members of glandular kallikrein gene family, yet only gamma subu...
journal_title：Neurochemical research
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更新日期：1991-06-01 00:00:00
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journal_title：Neurochemical research
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更新日期：2015-06-01 00:00:00
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journal_title：Neurochemical research
pub_type： 杂志文章
doi：10.1007/s11064-019-02728-1
更新日期：2019-04-01 00:00:00
abstract：:Brains of Alzheimer disease patients in early stages of dementia contain an increased 24(S)-hydroxycholesterol (cerebrosterol)/cholesterol ratio when compared to controls. In this study, effects of amyloid beta peptides and of racemic 24-hydroxycholesterol were evaluated in vitro on undepleted or cholesterol-depleted ...
journal_title：Neurochemical research
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更新日期：2008-03-01 00:00:00
