Abstract:
:In this review we focus on Charcot-Marie-Tooth (CMT) neuropathies and hereditary spastic paraplegias (HSPs). Although these diseases differ in whether they primarily affect the peripheral or central nervous system, both are genetically determined, progressive, long axonopathies that affect motor and sensory pathways. This commonality suggests that there might be similarities in the molecular pathology underlying these conditions, and here we compare the molecular genetics and cellular pathology of the two groups.
journal_name
Exp Neuroljournal_title
Experimental neurologyauthors
Timmerman V,Clowes VE,Reid Edoi
10.1016/j.expneurol.2012.01.010subject
Has Abstractpub_date
2013-08-01 00:00:00pages
14-25eissn
0014-4886issn
1090-2430pii
S0014-4886(12)00030-1journal_volume
246pub_type
杂志文章,评审abstract::RNA interference appears to have a great potential not only as an in vitro target validation, but also as a novel therapeutic strategy based on the highly specific and efficient silencing of a target gene. We hypothesize that MMP-9 siRNA can be effective as an MMP-9 protein inhibitor in a rat focal ischemia model. Mal...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/j.expneurol.2008.11.007
更新日期:2009-03-01 00:00:00
abstract::Apnea of prematurity (AoP) is associated with severe and repeated episodes of arterial oxygen desaturation (intermittent hypoxia - IH), which in turn increases the number of apneas. So far, there is no data addressing whether IH leads to sex-specific respiratory consequences, neither if drugs targeting AoP are more ef...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/j.expneurol.2019.112985
更新日期:2019-10-01 00:00:00
abstract::In 3-, 9-, 24-, and 30-month-old male rats (Fischer 344), the nuclear perimeter and the density and diameter of nuclear pore complexes in the granule cells of the dentate fascia were studied. Whereas the nuclear perimeter and the diameter of nuclear pore complexes did not change as a function of age, there was a signi...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/0014-4886(87)90314-1
更新日期:1987-03-01 00:00:00
abstract::The neuronal basis for neurological deficits in infantile hydrocephalus is poorly understood. Changes in the dendritic architecture of pyramidal cells of the auditory cortex have been measured at 21 days after birth in H-Tx rats. Tissue was prepared by the rapid Golgi method from hydrocephalic and control litter-mates...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1006/exnr.1996.0161
更新日期:1996-10-01 00:00:00
abstract::Neuropathological studies of human traumatic brain injury (TBI) cases have described amyloid plaques acutely after a single severe TBI, and tau pathology after repeat mild TBI (mTBI). This has helped drive the hypothesis that a single moderate to severe TBI increases the risk of developing late-onset Alzheimer's disea...
journal_title:Experimental neurology
pub_type: 杂志文章,评审
doi:10.1016/j.expneurol.2015.06.015
更新日期:2016-01-01 00:00:00
abstract::Neonatal stroke is increasingly recognized in preterm and term infants but the ability to study this condition has been limited by the technical challenges in developing suitable animal models. In the current study we report the use of transient filament middle cerebral artery occlusion for 1.5 h in 10-day-old rat pup...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/j.expneurol.2006.06.004
更新日期:2006-12-01 00:00:00
abstract::Tuberoinfundibular peptide of 39 residues (TIP39) synthesizing neurons at the caudal border of the thalamus and in the lateral pons project to areas rich in its receptor, the parathyroid hormone 2 receptor (PTH2R). These areas include many involved in processing nociceptive information. Here we examined the potential ...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/j.expneurol.2010.08.004
更新日期:2010-11-01 00:00:00
abstract::The reduction of GABA-mediated postsynaptic inhibition tolerated before seizure initiation was assessed using the GABA-mediated, disynaptic, inhibitory pathway from the ipsilateral vestibular nerve to trochlear motoneurons in pentobarbital-anesthetized cats. The amplitude of intracellularly recorded IPSPs or the corre...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/0014-4886(83)90284-4
更新日期:1983-05-01 00:00:00
abstract::Disease-modifying treatments remain an unmet medical need in Parkinson's disease (PD). Such treatments can be operationally defined as interventions that slow down the clinical evolution to advanced disease milestones. A treatment may achieve this outcome by either inhibiting primary neurodegenerative events ("neuropr...
journal_title:Experimental neurology
pub_type: 杂志文章,评审
doi:10.1016/j.expneurol.2017.10.001
更新日期:2017-12-01 00:00:00
abstract::Synaptic reorganization of mossy fibers following kainic acid (KA) administration has been reported to contribute to the formation of recurrent excitatory circuits, resulting in an epileptogenic state. It is unclear, however, whether KA-induced mossy fiber sprouting results from neuronal cell loss or the seizure activ...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1006/exnr.1999.7251
更新日期:2000-01-01 00:00:00
abstract::A cerebrospinal fluid (CSF) to brain exchange has been postulated for lipid-soluble and small molecular substances and has led to nearly 100 attempts per year to produce central effects via intrathecal injections. With few exceptions, however, modern neurological practice has avoided this approach because of its demon...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/0014-4886(84)90192-4
更新日期:1984-11-01 00:00:00
abstract::Excitotoxic mechanisms may play a critical role in the pathophysiology of several neurological and psychiatric diseases. Excitatory amino acid receptor antagonists are therefore of great therapeutic interest, but untoward side effects often prevent their clinical use. Targeting the glycine coagonist site of the (NMDA)...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1006/exnr.1999.7284
更新日期:2000-05-01 00:00:00
abstract::Urotensin-II (U-II) is a cyclic peptide identified recently in many mammalian species including man. U-II and its receptor are expressed in the central nervous system, in the cardiovascular system and in other peripheral tissues. Although this peptide has been reported initially to be a potent vasoconstrictor, increas...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/j.expneurol.2007.12.004
更新日期:2008-04-01 00:00:00
abstract::A sample of 504 single neurons isolated in three curvilinear arrays of 10 closely spaced tracks in primary somatosensory and in pericruciate sensorimotor cortex was studied in two awake, restrained domestic cats. Modality sensitivity and receptive field size and location were assessed for each neuron, along with respo...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/0014-4886(90)90065-z
更新日期:1990-01-01 00:00:00
abstract::Mutations in the human NOTCH3 gene cause cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), but the pathogenesis of CADASIL has remained unclear. Recently, endocytosis of the Notch ectodermal domain into ligand-expressing cells, called transendocytosis, has come to be...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/j.expneurol.2011.10.020
更新日期:2012-01-01 00:00:00
abstract::Rats typically display self-mutilation (autotomy) of a paw that has been denervated by transection of the sciatic and saphenous nerves. The cause of autotomy, however, is not known. It may be due to hyperesthesia (comparable to that seen in humans after peripheral nerve injury) or anesthesia (as an attempt to shed an ...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/0014-4886(86)90311-0
更新日期:1986-06-01 00:00:00
abstract::Excessive synchronization of neuronal activity at around 20 Hz is a common finding in the basal ganglia of patients with untreated Parkinson's disease (PD). Correlative evidence suggests, but does not prove, that this spontaneous activity may contribute to slowness of movement in this condition. Here we investigate wh...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/j.expneurol.2007.01.027
更新日期:2007-05-01 00:00:00
abstract::Mitochondria play a pivotal role in the cascade of events associated with cell death pathways that are involved with several forms of neurodegeneration. Recent findings show that in the Bax/Bak-dependent pathway of apoptosis, the release of cytochrome c from mitochondria is a consequence of two carefully coordinated e...
journal_title:Experimental neurology
pub_type: 杂志文章,评审
doi:10.1016/j.expneurol.2009.05.021
更新日期:2009-08-01 00:00:00
abstract::Adenoviral-mediated gene transfer to the caudate nucleus of Macaca mulatta was accomplished using stereotactic injection of two distinct recombinant Ad5 vectors containing the gene for Escherichia coli beta-galactosidase and the cDNA for rat hypoxanthine-guanine phosphoribosylphosphotransferase (HPRT), respectively. M...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1006/exnr.1994.1028
更新日期:1994-02-01 00:00:00
abstract::SCI is followed by dramatic upregulation of chondroitin sulfate proteoglycans (CSPGs) which limit axonal regeneration, oligodendrocyte replacement and remyelination. The recent discovery of the specific CSPGs signaling receptor protein tyrosine phosphatase sigma (RPTPσ) provided an opportunity to refine the therapeuti...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/j.expneurol.2018.08.003
更新日期:2018-11-01 00:00:00
abstract::Both alpha- and beta-adrenergic receptors (ARs) are involved in the facilitation of the monosynaptic jaw-closing reflex in the trigeminal motor nucleus (MoV) caused by norepinephrine (NE). The amplitude of muscle spindle afferent-evoked EPSPs in masseter motor neurons is 65% greater when noradrenergic axons to the mot...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/0014-4886(91)90038-e
更新日期:1991-11-01 00:00:00
abstract::Functional relations between hippocampal lamellae, the modular units arranged in parallel in the hippocampal formation, were studied in the guinea pig in experiments of evoked field potential analysis. Perforant path volleys, synaptically elicited by stimulation of the dorsal hippocampal commissure, were used to activ...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/0014-4886(83)90080-8
更新日期:1983-12-01 00:00:00
abstract::Caloric restriction (CR) is a daily reduction of total caloric intake without a decrease in micronutrients or disproportionate reduction of any one dietary component. CR can increase lifespan reliably in a wide range of species and appears to counteract some aspects of the aging process throughout the body. The effect...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/j.expneurol.2008.01.016
更新日期:2008-05-01 00:00:00
abstract::One of the primary hallmarks of glutamate excitotoxicity is degradation of the neuronal cytoskeleton. Using a tissue culture approach, we have investigated the relationship between excitotoxicity and cytoskeletal degradation within axons, with particular reference to the axon specific neurofilament proteins. Neurofila...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/j.expneurol.2005.01.005
更新日期:2005-06-01 00:00:00
abstract::Exposure to early life stress (ELS), such as childhood abuse and neglect is a well established major risk factor for developing psychiatric and behavioral disorders later in life. Both prenatal and postnatal stressors have been shown to have a long-lasting impact on adult pathological states where the type and timing ...
journal_title:Experimental neurology
pub_type: 杂志文章,评审
doi:10.1016/j.expneurol.2014.09.001
更新日期:2015-06-01 00:00:00
abstract::We recently described an experimental model of spinal cord contusive injury in the rat in which a modified weight-drop apparatus is used to generate reproducible mild, moderate, or severe final functional deficit. We now report several inexpensive alternatives for portions of the original apparatus which allow the pro...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/0014-4886(87)90162-2
更新日期:1987-02-01 00:00:00
abstract::We administered tetrahydroaminoacridine (THA), a cholinesterase inhibitor, to rats with bilateral nucleus basalis magnocellularis lesions and measured their performance in a spatial learning task. The subjects, 34 male Fischer-344 rats, received bilateral excitotoxic NBM lesions; 10 other rats served as unlesioned con...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/0014-4886(90)90126-d
更新日期:1990-06-01 00:00:00
abstract::Hydrocephalic mutant mice and matched siblings at different ages were used to measure the pressure and the resistance to drainage of the cerebrospinal fluid (CSF) from the lateral ventricles and from the cisterna magna with glass micropipets. The resting CSF pressure in normal mice increased between 1 to 2 and 4 to 8 ...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/0014-4886(85)90049-4
更新日期:1985-10-01 00:00:00
abstract::The monoclonal antibody Py has previously been shown to be a useful marker for subpopulations of neurons in the rat brain. However, the distribution of Py immunoreactivity in other regions of the CNS and PNS is not known. Here, we present a light and electron microscopic investigation into the distribution of Py immun...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1006/exnr.1997.6509
更新日期:1997-07-01 00:00:00
abstract::After spinal cord injury, proteoglycans with growth-inhibitory glycosaminoglycan (GAG-) side chains in scar tissue limit spontaneous axonal sprouting/regeneration. Interventions that reduce scar-related inhibition facilitate an axonal growth response and possibly plasticity-based spinal cord repair. Xylosyltransferase...
journal_title:Experimental neurology
pub_type: 杂志文章
doi:10.1016/j.expneurol.2012.06.006
更新日期:2012-09-01 00:00:00