Abstract：
:Familial dysautonomia (FD) is a sensory and autonomic neuropathy that affects the development and survival of sensory, sympathetic, and some parasympathetic neurons. It is autosomally inherited and occurs almost exclusively among individuals of Ashkenazi Jewish descent. The pathological and clinical manifestations of FD have been extensively studied and therapeutic modalities have, until recently, focused primarily on addressing the symptoms experienced by those with this fatal disorder. The primary FD-causing mutation is an intronic nucleotide substitution that alters the splicing of the IKBKAP-derived transcript. Recent efforts have resulted in the development of new therapeutic modalities that facilitate the increased production of the correctly spliced transcript and mitigate the symptoms of those with FD. Furthermore, the recent demonstration of the reduced presence of monoamine oxidase A in cells and tissues of individuals with FD has provided new insight into the cause of hypertensive crises experienced by these patients.
journal_name
Neuromolecular Medjournal_title
Neuromolecular medicineauthors
Rubin BY,Anderson SLdoi
10.1007/s12017-007-8019-5subject
Has Abstractpub_date
2008-01-01 00:00:00pages
148-56issue
3eissn
1535-1084issn
1559-1174journal_volume
10pub_type
杂志文章,评审abstract：:Clinacanthus nutans Lindau (C. nutans), commonly known as Sabah Snake Grass in southeast Asia, is widely used in folk medicine due to its analgesic, antiviral, and anti-inflammatory properties. Our recent study provided evidence for the regulation of cytosolic phospholipase A2 (cPLA2) mRNA expression by epigenetic fac...
journal_title：Neuromolecular medicine
pub_type： 杂志文章
doi：10.1007/s12017-016-8404-z
更新日期：2016-09-01 00:00:00
abstract：:Spinocerebellar ataxia type 10 (SCA10) is an autosomal dominant neurodegenerative disorder characterized by progressive cerebellar ataxia and epilepsy. The disease is caused by a pentanucleotide ATTCT expansion in intron 9 of the ATXN10 gene on chromosome 22q13.3. SCA10 has shown a geographical distribution throughout...
journal_title：Neuromolecular medicine
pub_type： 杂志文章
doi：10.1007/s12017-017-8464-8
更新日期：2017-12-01 00:00:00
abstract：:Danshensu (DSS) and tetramethylpyrazine (TMP) are active ingredients of Salvia miltiorrhiza Bge. and Ligusticum chuanxiong Hort that are widely used in oriental medicine. Structural combination of compounds with known biological activity may lead to the formation of a molecule with multiple properties or new function ...
journal_title：Neuromolecular medicine
pub_type： 杂志文章
doi：10.1007/s12017-016-8399-5
更新日期：2016-12-01 00:00:00
abstract：:The present study demonstrates the efficacies of synthetic 1,8-cineole and an 1,8-cineole-rich supercritical carbon dioxide (SC-CO2) extract of small cardamom seeds in preventing oligomerization of amyloid beta peptide (Aβ42) and inhibiting iron-dependent oxyradical production in vitro. The oligomerization of Aβ42 was...
journal_title：Neuromolecular medicine
pub_type： 杂志文章
doi：10.1007/s12017-019-08574-2
更新日期：2020-03-01 00:00:00
abstract：:Parkinson' disease (PD) is characterized by motor symptoms including bradykinesia, resting tremor, postural instability, and rigidity and non-motor symptoms such as cognitive impairment, sleep disorder, and depression. Neuroinflammation has been recently implicated in pathophysiology of both motor and non-motor sympto...
journal_title：Neuromolecular medicine
pub_type： 杂志文章
doi：10.1007/s12017-018-8499-5
更新日期：2018-09-01 00:00:00
abstract：:Functional electrical stimulation (FES) has been widely adopted to elicit muscle contraction in rehabilitation training after spinal cord injury (SCI). Conventional FES modalities include stimulations coupled with rowing, cycling, assisted walking and other derivatives. In this review, we studied thirteen clinical rep...
journal_title：Neuromolecular medicine
pub_type： 杂志文章,评审
doi：10.1007/s12017-019-08589-9
更新日期：2020-12-01 00:00:00
abstract：BACKGROUND:Scutellarin, an herbal compound, can effectively suppress the inflammatory response in activated microglia/brain macrophage(AM/BM) in experimentally induced cerebral ischemia; however, the underlying mechanism for this has not been fully clarified. We sought to elucidate if scutellarin would exert its anti-i...
journal_title：Neuromolecular medicine
pub_type： 杂志文章
doi：10.1007/s12017-019-08582-2
更新日期：2020-06-01 00:00:00
abstract：:Accumulating studies have suggested the important role of microRNA (miRNA) in ischemic cerebral infarction. However, little is known of the modifying effect of miR-5787, a newly found miRNA, in ischemic cerebral infarction. We aim to elucidate the effect and underlying molecular mechanism of miR-5787 in the pathogenes...
journal_title：Neuromolecular medicine
pub_type： 杂志文章
doi：10.1007/s12017-020-08628-w
更新日期：2020-11-09 00:00:00
abstract：:The original version of this article unfortunately contained a mistake. Gary S. Laco should not be listed as an author in the author group. ...
journal_title：Neuromolecular medicine
pub_type： 杂志文章,已发布勘误
doi：10.1007/s12017-020-08593-4
更新日期：2020-06-01 00:00:00
abstract：:Rett syndrome is a severe neurodevelopmental disorder, almost exclusively affecting females and characterized by a wide spectrum of clinical manifestations. Both the classic and atypical forms of Rett syndrome are primarily due to mutations in the methyl-CpG-binding protein 2 (MECP2) gene. Mutations in the X-linked cy...
journal_title：Neuromolecular medicine
pub_type： 杂志文章
doi：10.1007/s12017-012-8212-z
更新日期：2013-03-01 00:00:00
abstract：:Epidemiological studies demonstrate that pain frequently occurs comorbid with depression. Gentiopicroside (Gent) is a secoiridoid compound isolated from Gentiana lutea that exhibits analgesic properties and inhibits the expression of GluN2B-containing N-methyl-D-aspartate (NMDA) receptors in the anterior cingulate cor...
journal_title：Neuromolecular medicine
pub_type： 杂志文章
doi：10.1007/s12017-013-8280-8
更新日期：2014-06-01 00:00:00
abstract：:Neuronal diseases, including retinal disorders, stroke, Alzheimer's disease, Parkinson's disease and spinal cord injury, affect a large number of people worldwide and cause heavy social and economic burdens. Although many efforts have been made by scientists and clinicians to develop novel drug and healthcare strategi...
journal_title：Neuromolecular medicine
pub_type： 杂志文章,评审
doi：10.1007/s12017-016-8393-y
更新日期：2016-09-01 00:00:00
abstract：:The cerebral ischemia is one of the most common diseases in the central nervous system that causes progressive disability or even death. In this connection, the inflammatory response mediated by the activated microglia is believed to play a central role in this pathogenesis. In the event of brain injury, activated mic...
journal_title：Neuromolecular medicine
pub_type： 杂志文章,评审
doi：10.1007/s12017-016-8394-x
更新日期：2016-09-01 00:00:00
abstract：:Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that primarily affects motor neurons and descending motor tracts of the CNS. We have evaluated the CNS of a murine model of familial ALS based on the over-expression of mutant human superoxide dismutase (mSOD; G93A) using magnetic resonance microscopy ...
journal_title：Neuromolecular medicine
pub_type： 杂志文章
doi：10.1007/s12017-007-8002-1
更新日期：2007-01-01 00:00:00
abstract：:Neurons and especially their synapses often project long thin processes that can invaginate neighboring neuronal or glial cells. These "invaginating projections" can occur in almost any combination of postsynaptic, presynaptic, and glial processes. Invaginating projections provide a precise mechanism for one neuron to...
journal_title：Neuromolecular medicine
pub_type： 杂志文章,评审
doi：10.1007/s12017-015-8358-6
更新日期：2015-09-01 00:00:00
abstract：:Neurodegeneration is the main pathological correlate of accumulating disability in progressive stages of Multiple Sclerosis (MS), but both histologic and imaging studies detect significant tissue loss even in early disease. These observations raise the question as to whether neurodegeneration in MS is a primary mechan...
journal_title：Neuromolecular medicine
pub_type： 杂志文章,评审
doi：10.1385/NMM:3:3:147
更新日期：2003-01-01 00:00:00
abstract：:The X-linked form of Charcot-Marie-Tooth disease (CMT1X) is the second most common molecularly designated form of hereditary motor and sensory neuropathy. The clinical phenotype is characterized by progressive distal muscle atrophy and weakness, areflexia, and variable sensory abnormalities. Affected males have modera...
journal_title：Neuromolecular medicine
pub_type： 杂志文章,评审
doi：10.1385/nmm:8:1-2:107
更新日期：2006-01-01 00:00:00
abstract：:Vitamin D deficiency is suggested to be associated with Parkinson's disease (PD). Our aim was to investigate the serum 25-hydroxyvitamin D3 (25OHD) levels of PD patients in Turkish cohort, to investigate any association of vitamin D binding protein (GC) genotypes with PD due to the significant role of GC in vitamin D ...
journal_title：Neuromolecular medicine
pub_type： 杂志文章
doi：10.1007/s12017-016-8415-9
更新日期：2017-03-01 00:00:00
abstract：:Millions of individuals with active TB do not receive recommended treatments, and instead may use botanicals, or use botanicals concurrently with established treatments. Many botanicals protect against oxidative stress, but this can interfere with redox-dependent activation of isoniazid and other prodrugs used for pro...
journal_title：Neuromolecular medicine
pub_type： 杂志文章,评审
doi：10.1007/s12017-016-8402-1
更新日期：2016-09-01 00:00:00
abstract：:T-cells directed to self-antigens ("autoimmune" T-cells) have traditionally been perceived as tending to attack the body's own tissues, and likely to exert their destructive effects unless they undergo deletion in the thymus during ontogeny. Naturally occurring CD4+CD25+ regulatory T-cells were viewed as thymus-derive...
journal_title：Neuromolecular medicine
pub_type： 杂志文章,评审
doi：10.1385/NMM:7:3:197
更新日期：2005-01-01 00:00:00
abstract：:We have previously reported that mild traumatic brain injury (mTBI) induced cognitive deficits as well as apoptotic changes in the brains of mice. Apoptosis may be caused by severe, prolonged accumulation of misfolded proteins, and protein aggregation in the endoplasmic reticulum (ER stress). In an additional study, w...
journal_title：Neuromolecular medicine
pub_type： 杂志文章
doi：10.1007/s12017-015-8340-3
更新日期：2015-03-01 00:00:00
abstract：:Selective degeneration and death of motor neurons in SOD1 mutant-mediated amyotrophic lateral sclerosis (ALS) is accompanied by axonal disorganization and reduced slow axonal transport in the three most frequently used mouse models of mutant SOD1-mediated ALS. To test whether suppression of axonal degeneration (freque...
journal_title：Neuromolecular medicine
pub_type： 杂志文章
doi：10.1385/NMM:5:3:193
更新日期：2004-01-01 00:00:00
abstract：:There is increasing evidence implicating apoptosis-mediated cell death in the pathogenesis of neurodegenerative diseases. One important event in the apoptotic cascade is the release of cytochrome c by mitochondria into the cytoplasm, activating caspase-9, leading to the subsequent activation of downstream executioner ...
journal_title：Neuromolecular medicine
pub_type： 杂志文章
doi：10.1385/NMM:1:3:183
更新日期：2002-01-01 00:00:00
abstract：:More than 30 genetic causes have been identified for the inherited neuropathies collectively referred to as Charcot-Marie-Tooth (CMT) disease. Previous therapies for CMT were limited to traditional approaches such as rehabilitation medicine, ambulation aids, and pain management. Identification of the genes causing CMT...
journal_title：Neuromolecular medicine
pub_type： 杂志文章
doi：10.1385/nmm:8:1-2:255
更新日期：2006-01-01 00:00:00
abstract：:Despite the significant amount of experimental data available on trehalose, the molecular mechanism responsible for its intracellular stabilising properties has not emerged yet. The repair of cellular homeostasis in many protein-misfolding diseases by trehalose is credited to the disaccharide being an inducer of autop...
journal_title：Neuromolecular medicine
pub_type： 杂志文章
doi：10.1007/s12017-013-8275-5
更新日期：2014-06-01 00:00:00
abstract：:Reperfusion after cerebral ischemia causes additional ischemic injuries due to sudden recovery of blood supply. It usually produces excessive reactive species, mitochondrial dysfunction, oxidative stress, and cell apoptosis. Our study is designed to examine the role of miR-421 antagomir in cerebral ischemia/reperfusio...
journal_title：Neuromolecular medicine
pub_type： 杂志文章
doi：10.1007/s12017-020-08600-8
更新日期：2020-09-01 00:00:00
abstract：:Phospholipids are the main constituents of brain membranes. Formation of new membranes requires that uridine, the omega-3 polyunsaturated fatty acids such as docosahexaenoic acid (DHA), and choline, the three circulating precursors of major phospholipids, interact via the Kennedy pathway. Supplementation of laboratory...
journal_title：Neuromolecular medicine
pub_type： 杂志文章
doi：10.1007/s12017-016-8414-x
更新日期：2016-09-01 00:00:00
abstract：:Hypothalamic neuropeptide Y (NPY) and superoxide dismutase (SOD) have been reported to participate in the regulation of appetite-suppressing effect of phenylpropanolamine (PPA), a sympathomimetic agent. This study explored whether Y1 receptor (Y1R) and/or Y5 receptor (Y5R) was involved in this regulation. Wistar rats ...
journal_title：Neuromolecular medicine
pub_type： 杂志文章
doi：10.1007/s12017-012-8206-x
更新日期：2013-03-01 00:00:00
abstract：:The wide use of serotonin reuptake inhibitors and serotonin receptor agonists in anxiety disorders has suggested a key role for the modulatory neurotransmitter in anxiety. However, serotonin's specific role is still uncertain. This article reviews the literature concerning how and where serotonergic agents modulate an...
journal_title：Neuromolecular medicine
pub_type： 杂志文章,评审
doi：10.1385/NMM:5:1:027
更新日期：2004-01-01 00:00:00
abstract：:In addition to myelin loss and oligodendrocyte injury, axonal damage is a major cause of irreversible neurological disability in multiple sclerosis (MS). A series of studies have demonstrated that Rho kinase (ROCK) is involved in synaptic plasticity of neurons. Here, we found that ROCK activity in MS serum was elevate...
journal_title：Neuromolecular medicine
pub_type： 杂志文章
doi：10.1007/s12017-015-8374-6
更新日期：2015-12-01 00:00:00
