Abstract：
:Zellweger syndrome (ZS) is a neonatal-lethal genetic disease that affects all tissues, and features neuropathology that involves primary developmental defects as well as neurodegeneration. Neuropathological changes include abnormal neuronal migration affecting the cerebral hemispheres, cerebellum and inferior olivary complex, abnormal Purkinje cell arborisation, demyelination and post-developmental neuronal degeneration. ZS is caused by mutations in peroxisome biogenesis, or PEX, genes which lead to defective peroxisome biogenesis and the resultant loss of peroxisomal metabolic function. The molecular and cellular bases of ZS neuropathology are still not completely understood. Attempts to explain the neuropathogenesis have implicated peroxisomal metabolic dysfunction, and more specifically the loss of peroxisomal products, such as plasmalogens and docosahexaenoic, and the accumulation of peroxisomal substrates, such as very-long-chain-fatty acids. In this review, consideration is also given to recent findings that implicate other candidate pathogenetic factors, such as mitochondrial dysfunction, oxidative stress, protein misfolding, aberrant cell signalling, and inflammation - factors that have also been identified as important in the pathogenesis of other neurological diseases.
journal_name
Neurochem Intjournal_title
Neurochemistry internationalauthors
Crane DIdoi
10.1016/j.neuint.2014.02.007subject
Has Abstractpub_date
2014-04-01 00:00:00pages
1-8eissn
0197-0186issn
1872-9754pii
S0197-0186(14)00037-0journal_volume
69pub_type
杂志文章,评审abstract：:The synthesis of neurotransmitter glutamate in brain is mainly carried out by glutaminase enzymes. This synthesis must be exquisitely regulated because of its harmful potential giving rise to excitotoxic damage. It is noteworthy that two glutaminase isozymes coded by different genes are expressed in the brain of mamma...
journal_title：Neurochemistry international
pub_type： 杂志文章,评审
doi：10.1016/j.neuint.2009.02.021
更新日期：2009-07-01 00:00:00
abstract：:Human atrial natriuretic peptide (Ser 99-Tyr 126) was rapidly degraded by both choroid plexus and hypothalamic membranes with a complex pattern of cleavage. The use of protease inhibitors allowed a preliminary characterization of the enzymes involved in the hydrolysis of the Ser-Phe and Phe-Arg bonds of iodine-labelle...
journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/0197-0186(88)90176-3
更新日期：1988-01-01 00:00:00
abstract：:Alzheimer disease (AD) is the most common form of adult dementia. Its pathological hallmarks are synaptic degeneration, deposition of amyloid plaques and neurofibrillary tangles, leading to neuronal loss. A few hypotheses have been proposed to explain AD pathogenesis. The beta-amyloid (Abeta) and hyperphosphorylated t...
journal_title：Neurochemistry international
pub_type： 杂志文章,评审
doi：10.1016/j.neuint.2007.10.002
更新日期：2008-03-01 00:00:00
abstract：:The brain uses long-chain fatty acids (LCFAs) to a negligible extent as fuel for the mitochondrial energy generation, in contrast to other tissues that also demand high energy. Besides this generally accepted view, some studies using cultured neural cells or whole brain indicate a moderately active mitochondrial β-oxi...
journal_title：Neurochemistry international
pub_type： 杂志文章,评审
doi：10.1016/j.neuint.2017.03.018
更新日期：2017-10-01 00:00:00
abstract：:This study aimed to elucidate locomotor activity changes in 6-hydroxydopamine (6-OHDA) induced Parkinson's disease (PD) and investigate the possible beneficial effects of melatonin on altered levels of locomotor activity, cyclooxygenase (COX), prostaglandin E2 (PGE2), nuclear factor kappa-B (NF-κB), nitrate/nitrite an...
journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/j.neuint.2014.09.005
更新日期：2014-12-01 00:00:00
abstract：:The relative abilities of a series of local anesthetics in which either or both the ester oxygens had been replaced with sulfur or selenium to block axonal or synaptic preparations are compared with their abilities to displace (3)H-nicotine from lobster axon plasma membrane fragments. The relative affinities of a seri...
journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/0197-0186(80)90011-x
更新日期：1980-01-01 00:00:00
abstract：:Excitotoxicity induced by excessive glutamate has been implicated in many brain disorders. Xanthohumol is a natural product derived from hops (Humulus lupulus L.), which is reported to have glutamate release-inhibiting activity. However, it is unknown whether xanthohumol has protective effects against glutamate-induce...
journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/j.neuint.2019.104629
更新日期：2020-02-01 00:00:00
abstract：:Viral infections are implicated in the onset and promotion of autoimmunity in genetically predisposed individuals. In this study, immune response patterns to herpes viruses were compared in aquaporin 4 (AQP4) antibody positive central nervous system (CNS) autoimmunity and multiple sclerosis (MS). Serum samples of pati...
journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/j.neuint.2010.08.003
更新日期：2010-11-01 00:00:00
abstract：:Monosialoganglioside (GM1) is a glycosphingolipid present in most cell membranes that displays antioxidant and neuroprotective properties. It has been recently described that GM1 induces vasodilation. However, the mechanisms underlying GM1-induced vasodilation were not evaluated to date. Therefore, in this study we in...
journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/j.neuint.2008.07.011
更新日期：2008-12-01 00:00:00
abstract：:To identify whether the process of apoptosis bears a topographic relationship to selected aspects of Alzheimer's disease (AD) pathology, we used an in situ nick translation method (TUNEL) to map DNA fragmentation in hippocampal sections immunostained for abnormally phosphorylated tau, which exists in the neurofibrilla...
journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/s0197-0186(96)00158-1
更新日期：1997-08-01 00:00:00
abstract：:Immunoglobulin G (IgG) antibodies against myelin oligodendrocyte glycoprotein (MOG) are detected in the serum of some patients with demyelinating diseases. These patients are known to show repeated clinical episodes of inflammatory demyelinating attacks in the central nervous system. Although the associated pathogenic...
journal_title：Neurochemistry international
pub_type： 杂志文章,评审
doi：10.1016/j.neuint.2018.10.016
更新日期：2019-11-01 00:00:00
abstract：:The mobilization of inositol triphosphate ip3 by N-methyl D-aspartate (NMDA) and kainate, two excitatory amino acid EAA receptor agonists, was studied in cultured chick retina cells as a function of culture differentiation. Kainate (EC50 = 30 microM) stimulated from 6 to 9-fold the production of [3H]ip3 between E8C3 (...
journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/0197-0186(94)00143-i
更新日期：1995-04-01 00:00:00
abstract：:We examined the intracellular delivery of Pep-1-cargo protein against transient ischemic damage in the hippocampal CA1 region in gerbils. For this study, we introduced green fluorescent protein (GFP) and constructed Pep-1-GFP protein. At 12h after Pep-1-GFP treatment, GFP fluorescence was shown in almost CA1 pyramidal...
journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/j.neuint.2007.08.013
更新日期：2008-03-01 00:00:00
abstract：:The neuromodulator adenosine, has been shown to have the highest density of central uptake sites in the nucleus tractus solitarius in the dorsal brain stem. The nucleus tractus solitarius is involved in the central regulation of reflex cardiovascular activity suggesting that adenosine may also be involved in central c...
journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/0197-0186(94)90065-5
更新日期：1994-09-01 00:00:00
abstract：:Age-related and aged memory deficit changes in Nestin-immunoreactive (Nestin-IR) neurons were studied following recent evidence of distinct Nestin-IR neurons within adult rat basal forebrain. Morris water maze task assessed spatial learning capacity of 3- and 24-month rats (aged-impaired and aged-unimpaired groups). N...
journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/j.neuint.2008.08.006
更新日期：2008-12-01 00:00:00
abstract：:Zinc transporter 3 (ZNT3) has been shown to transport zinc ions from the cytosol into presynaptic vesicles in the mammalian brain. Several studies have stated that the zinc ion containing synaptic vesicles of zinc-enriched neurons (ZEN) are loaded with ZNT3 proteins in their membranes. This fact makes it possible to t...
journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/j.neuint.2008.02.008
更新日期：2008-06-01 00:00:00
abstract：:In the past few years, several studies have demonstrated in the rat subcommissural organ the presence of nerve endings and modified ependymocytes showing an uptake of [(3)H]GABA. The present work was performed to demonstrate in this cerebral zone the possibility of a GABA synthesis by the immunohistochemical localizat...
journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/0197-0186(83)90105-5
更新日期：1983-01-01 00:00:00
abstract：:Parkinson's disease (PD), a progressive neurodegenerative disorder, has long been associated with mitochondrial dysfunction in both sporadic and familial forms of the disease. Mitochondria are crucial for maintaining cellular homeostasis, and their dysfunction is detrimental to dopaminergic neurons. These neurons are ...
journal_title：Neurochemistry international
pub_type： 杂志文章,评审
doi：10.1016/j.neuint.2019.104495
更新日期：2019-10-01 00:00:00
abstract：:Human glutamate dehydrogenase (GDH) gene was fused with a gene fragment encoding the nine amino acid (RKKRRQRRR) protein transduction domain of human immunodeficiency virus TAT protein in bacterial expression vector to produce genetic in-frame TAT-GDH fusion protein. The TAT-GDH protein can enter PC12 cells efficientl...
journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/s0197-0186(01)00138-3
更新日期：2002-07-01 00:00:00
abstract：:The presence of acetylcholinesterase (AChE) mRNA and activity in the tissues and cells involved in immune responses prompted us to investigate the level and pattern of AChE components in spleen. AChE activity was higher in mouse spleen (0.46 +/- 0.13 micromol of acetylthiocholine split per hour and per mg protein) tha...
journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/j.neuint.2003.11.019
更新日期：2004-07-01 00:00:00
abstract：:Radial glial cells play a significant role in the repair of spinal cord injuries as they exert critical role in the neurogenesis and act as a scaffold for neuronal migration. Our previous study showed that mature astrocytes of spinal cord can undergo a de-differentiation process and further transform into pluripotenti...
journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/j.neuint.2011.08.021
更新日期：2011-12-01 00:00:00
abstract：:In the present investigation we address the question of whether one of the responses to increased neuronal activity is a modification of the expression of the different subunits of alpha-amino-3-hydroxy-5-methyl-4-isoxazole propionate (AMPA)-selective glutamate receptors (GluR-1, GluR-2, GluR-3). Thus, we used two dif...
journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/0197-0186(94)90144-9
更新日期：1994-10-01 00:00:00
abstract：:Sodium- and energy-dependent accumulation of [(3)H]l-glutamic acid (Glu) into rat cerebral cortical slices was inhibited by relatively high concentrations (40-100 ?M) of calmodulin antagonists, such as N-(6-aminohexyl)-1-naphthalenesulfonamide and N-(6-aminohexyl)-5-chloro-1-naphthalenesulfonamide, in concentration-de...
journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/0197-0186(89)90071-5
更新日期：1989-01-01 00:00:00
abstract：:Antibodies to electrolectin, a lectin endogenous to embryonic skeletal muscle, have been used to study the distribution of lectin during myogenesis in L(6) cells by using immunohistofluorescence microscopy. Evidence for both surface and cytoplasmic forms of lectin is presented. Cytoplasmic lectin does not appear to be...
journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/0197-0186(80)90041-8
更新日期：1980-01-01 00:00:00
abstract：:Selective dopaminergic neurotoxicity induced by 1-methyl-4-phenylpyridine (MPP+) is believed to be due to the transmembrane uptake by the dopamine transporter and subsequent inhibition of mitochondrial complex I and/or production of free radicals. However, little is known about the molecular sequence of intracellular ...
journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/s0197-0186(99)00083-2
更新日期：1999-11-01 00:00:00
abstract：:Nucleotides can be released as signaling substances in the nervous system from both neural and glial cells. Their function is terminated by ecto-nucleotidases and sequential extracellular metabolism to the nucleoside. Recently considerable progress has been made in unraveling the molecular structure of an ecto-ATPase ...
journal_title：Neurochemistry international
pub_type： 杂志文章,评审
doi：10.1016/s0197-0186(97)00126-5
更新日期：1998-05-01 00:00:00
abstract：:The N-methyl-D-aspartate (NMDA) receptor is a subtype of ionotropic glutamate receptor that is involved in synaptic mechanisms of learning and memory, and mediates excitotoxic neuronal injury. In this study, we tested the hypothesis that NMDA receptor subunit gene expression is altered in cortex and hippocampus of OKA...
journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/j.neuint.2014.06.012
更新日期：2014-10-01 00:00:00
abstract：:The roles of cyclic AMP and calcium in the regulation of serotonin N-acetyltransferase (NAT) activity were studied in low density monolayer cultures of chick retinal photoreceptors and neurons. Photoreceptor-enriched retinal cell cultures were prepared from embryonic day 6 retinas and cultured for 6 days. NAT activity...
journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/0197-0186(95)00035-7
更新日期：1995-08-01 00:00:00
abstract：:Brain cortex synaptoneurosomes actively incorporated [14C]arachidonic acid (AA) into lipids. Serotonin (5-HT), at a concentration range of 10 microM-1 mM, significantly stimulates the incorporation of AA mainly into phosphatidylinositol (PI) of brain cortex synaptoneurosomes. The stimulation rate of AA incorporation b...
journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/0197-0186(94)90039-6
更新日期：1994-08-01 00:00:00
abstract：:The present study was designed to investigate the turnover rates of D1 dopamine receptors in the brain and retina of adult and aged rats. To this aim, we monitored the increase in the density of 3H-SCH 23390 binding sites after the administration of the irreversible antagonist N-ethoxycarbonyl-2-ethoxy-1,2 dihydroquin...
journal_title：Neurochemistry international
pub_type： 杂志文章
doi：10.1016/0197-0186(92)90233-h
更新日期：1992-03-01 00:00:00
