Abstract：
:Intranuclear inclusion bodies are a shared pathological feature of Huntington's disease (HD) and its transgenic mouse model. Using a panel of antibodies spanning the entire huntingtin molecule, we have investigated the pattern of immunoreactivity within the intranuclear inclusions in the frontal cortex and striatum of patients with HD. The intranuclear inclusions reacted with anti-ubiquitin and antibodies against the N-terminal portion of huntingtin (CAG53b, HP1), but not with HD1 and the 1C2 antibodies that detect the expanded polyglutamine tract nor the more C-terminal antibodies. However, the 1C2, HP1, CAG53b, and HD1 antibodies detected granular cytoplasmic deposits in cortical and striatal neurons that also contained intranuclear N-terminal huntingtin immunoreactivity. These data show a differential intracellular location of truncated huntingtin in the HD brain. Both the cytoplasmic and the nuclear aggregates of the protein fragments could be neurotoxic. The frequency of the cortical intranuclear inclusions correlated with the size of CAG expansion and was inversely related to the age at onset and death. No such correlations were detected for the striatum, which most likely reflects a more advanced neuronal loss accrued by the time of death.
journal_name
Exp Neuroljournal_title
Experimental neurologyauthors
Sieradzan KA,Mechan AO,Jones L,Wanker EE,Nukina N,Mann DMdoi
10.1006/exnr.1998.7005keywords:
subject
Has Abstractpub_date
1999-03-01 00:00:00pages
92-9issue
1eissn
0014-4886issn
1090-2430pii
S0014-4886(98)97005-4journal_volume
156pub_type
杂志文章abstract：:Globoid cell leukodystrophy (GLD, Krabbe Disease) is a lysosomal storage disease, resulting from the genetic deficiency of galactosylceramidase (GALC). This disease is marked by accumulation of the cytotoxic lipid psychosine (Psy). Psychosine is known to induce oxidative stress in cultured cells, and this stress can b...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2012.07.013
更新日期：2012-10-01 00:00:00
abstract：:Alpha-tyrosinated tubulin is a cytoskeletal protein that is involved in axonal growth and is considered a marker of neuronal plasticity in adult mammals. In adult rats, unilateral ablation of the left facial sensorimotor cortical areas induces degeneration of corticotrigeminal projections and marked denervation of the...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2008.05.010
更新日期：2008-09-01 00:00:00
abstract：:Downregulation in the astroglial glutamate transporter EAAT2 in amyotrophic lateral sclerosis (ALS) patients and mutant SOD1 mouse models of ALS is believed to contribute to the death of motor neurons by excitotoxicity. We previously reported that caspase-3 cleaves EAAT2 at a unique cleavage consensus site located in ...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2017.03.014
更新日期：2017-06-01 00:00:00
abstract：:Muscle disuse-induced changes in the cholinergic system of sciatic nerve, slow-twitch soleus (SOL), and fast-twitch extensor digitorum longus (EDL) muscles were studied in rats. Rats with hind limbs suspended for 2 to 3 weeks showed marked elevation in the activity of choline acetyltransferase in sciatic nerve (38%), ...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(85)90012-3
更新日期：1985-09-01 00:00:00
abstract：:Propentofylline is a xanthine derivative that has been known to protec t neurons against ischemia-induced damage. To assess its neuroprotective mechanisms, we examined the effect of propentofylline on microglial proliferation that is thought to play an important role in neuronal damage. We determined the proliferation...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.1996.0035
更新日期：1996-02-01 00:00:00
abstract：:The competence of neurons to regenerate depends on their ability to initiate a program of gene expression supporting growth and on the growth-permissive properties of glial cells in the distal stump of the injured nerve. Most studies on intrinsic molecular mechanisms governing peripheral nerve regeneration have focuss...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.1998.6886
更新日期：1998-10-01 00:00:00
abstract：:Whereas quite a number of previous reports have shown that the developing brain is vulnerable to protein deprivation, the mature CNS is usually considered resistant to this condition. To explore this assumption the present experimental model was built on groups of 2-month-old rats fed with a low-protein diet (8% casei...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(89)90081-2
更新日期：1989-02-01 00:00:00
abstract：:Impaired olfaction is an early symptom of Alzheimer disease (AD). This likely to reflect neurodegenerative processes taking place in basal telencephalic structures that mediate olfactory processing, including the anterior olfactory nucleus. Betaeta-amyloid (Abeta) accumulation in AD brain may relate to decline in soma...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2009.06.010
更新日期：2010-06-01 00:00:00
abstract：:As in Alzheimer's disease, brains of Guam Chamorros with amyotrophic lateral sclerosis (ALS) and Parkinsonism-dementia complex (PDC) contain intraneuronal-paired helical filaments composed of accumulated phosphorylated tau protein. Tau mRNA expression in rat neuronal cultures-normally modulated by glutamate-increases ...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.1998.6962
更新日期：1999-01-01 00:00:00
abstract：:Transgenic mice whose axons and Schwann cells express fluorescent chromophores enable new imaging techniques and augment concepts in developmental neurobiology. The utility of these tools in the study of traumatic nerve injury depends on employing nerve models that are amenable to microsurgical manipulation and gaugin...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2007.05.028
更新日期：2007-09-01 00:00:00
abstract：:Electromyograms from the flexor and extensor muscles of normal human elbows were simultaneously recorded during maximal-effort isokinetic movement at 15 degrees/s over the joint's full range of motion. The antagonist electromyogram was normalized with respect to its electromyogram when acting as agonist at maximal eff...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(88)90032-5
更新日期：1988-06-01 00:00:00
abstract：:Dopamine (DA) release and metabolism within the substantia nigra (SN) were studied in normal rats, rats with unilateral 6-hydroxydopamine (6-OHDA) lesions, and 6-OHDA-lesioned rats treated with glial cells line-derived neurotrophic factor (GDNF). Animals with > 99% DA depletions, as determined by apomorphine-induced c...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.1997.6571
更新日期：1997-09-01 00:00:00
abstract：:The possible pain inhibitory effects of periaqueductal gray (PAG) stimulation were investigated in cats anesthetized with Nembutal and immobilized with Flaxedil. Unitary responses evoked by electrical stimulation of the upper canine dentine and by cutaneous facial noxious and nonnoxious stimuli were recorded extracell...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(83)90155-3
更新日期：1983-07-01 00:00:00
abstract：:Pretreatment with the synthetic glucocorticoid dexamethasone prevents hypoxic-ischemic brain damage in 7-day-old neonatal rats. We presently characterize the response further by examining the effect of varying the age, the glucocorticoid, and the time of injection and by examining whether fasting can influence the res...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(95)90065-9
更新日期：1995-03-01 00:00:00
abstract：:We compared mitochondrial respiratory chain function, mitochondrial DNA (mtDNA) integrity, and oxidative stress levels in muscle, myoblasts, fibroblasts and cybrids, from 12 amyotrophic lateral sclerosis (ALS) patients with 28 control samples. Mitochondrial respiratory chain enzyme activities were normal in muscle, my...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2009.04.007
更新日期：2009-07-01 00:00:00
abstract：:Glial cell line-derived neurotrophic factor (GDNF) is a potent survival factor for nigrostriatal dopaminergic, central cholinergic, and motoneurons. GDNF also prevents the neuronal loss in experimental animal models for Parkinson's disease (PD). We have now investigated the GDNF gene for possible mutations in a group ...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.1998.6857
更新日期：1998-08-01 00:00:00
abstract：:The majority of human spinal cord injuries involve gray matter loss from the cervical or lumbar enlargements. However, the deficits that arise from gray matter damage are largely masked by the severe deficits due to associated white matter damage. We have developed a model to examine gray matter-specific deficits and ...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.1999.7016
更新日期：1999-03-01 00:00:00
abstract：:Here, we describe a bilateral cervical contusion model for mice. Adult female mice received graded bilateral contusion injuries at cervical level 5 (C5) using a commercially available impactor (the IH device). Three separate experiments were carried out to define conditions that produce impairments in forelimb functio...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2009.09.028
更新日期：2010-01-01 00:00:00
abstract：:Axons of retinal ganglion cells (RGCs) of adult cats were transected and regenerated by the transplantation of peripheral nerves. Two months after transplantation dendritic arbors of RGCs with regenerated axons (r-RGCs) were visualized with intracellular injections of horseradish peroxidase (HRP). The HRP-filled r-RGC...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.1996.6345
更新日期：1997-01-01 00:00:00
abstract：:Dopaminergic (DAergic) hypofunction in the medial prefrontal cortex (mPFC) has been implicated in the negative and cognitive symptoms of schizophrenia and is regulated by serotonergic (5-HTergic) neurons in the dorsal raphe nucleus (DRN). The lateral habenula (LHb) is a key element in controlling DRN 5-HT neurons. We ...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2019.05.005
更新日期：2019-08-01 00:00:00
abstract：:Synthesis of leukemia inhibitory factor (LIF) is increased in lesioned peripheral nerves and it is thought that this may cause increased expression of galanin (GAL) in axotomized dorsal root ganglia (DRG) neurons and also to promote axonal regeneration. We therefore compared effects of LIF and nerve growth factor (NGF...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.2001.7667
更新日期：2001-06-01 00:00:00
abstract：:Subarachnoid hemorrhage (SAH) is the most devastating form of stroke. Reducing neuronal apoptosis is an important countermeasure against early brain injury (EBI) after SAH. Recent evidence indicates that OX40-OX40L coupling is critical for cell survival and proliferation. Current study was performed to detect the role...
journal_title：Experimental neurology
pub_type： 杂志文章,评审
doi：10.1016/j.expneurol.2020.113179
更新日期：2020-04-01 00:00:00
abstract：:Estrogen has been suggested to be pro-epileptic by reducing GABA synthesis, resulting in increased spine density and a decreased threshold for seizures in the hippocampus, which, once they occur, are characterized by a dramatic spine loss in the affected brain areas. As considerable amounts of estradiol are synthesize...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2006.07.020
更新日期：2007-01-01 00:00:00
abstract：:Dopaminergic projections to the caudate putamen (CPu) involve fibers in the nigrostriatal pathway from the ipsilateral substantia nigra-pars compacta. Post-synaptic receptor populations on cells receiving this information are composed of both D1 and D2 dopamine receptor subtypes. In the present study, unilateral lesio...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.1993.1069
更新日期：1993-05-01 00:00:00
abstract：:The respiratory central pattern generator must respond to chemosensory cues to maintain oxygen (O2) and carbon dioxide (CO2) homeostasis in the blood and tissues. To do this, sensorial cells located in the periphery and central nervous system monitor the arterial partial pressure of O2 and CO2 and initiate respiratory...
journal_title：Experimental neurology
pub_type： 杂志文章,评审
doi：10.1016/j.expneurol.2016.05.036
更新日期：2017-01-01 00:00:00
abstract：:Repetitive transcranial magnetic stimulation (rTMS) of the dorsolateral prefrontal cortex is a relatively non-invasive technique with putative therapeutic effects in major depression. However, the exact neurophysiological basis of these effects needs further clarification. Therefore, we studied the impact of ten daily...
journal_title：Experimental neurology
pub_type： 临床试验,杂志文章
doi：10.1016/j.expneurol.2005.08.008
更新日期：2005-12-01 00:00:00
abstract：:The ability of striatal embryonic grafts to promote functional recovery on complex behavioral tasks depends on various factors, including the amount of striatal-like tissue within the grafts and the duration of post-graft training. However, how the innate paw bias of animals is affected by experience, or influences re...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2005.12.033
更新日期：2006-06-01 00:00:00
abstract：:beta-N-methylamino-l-alanine (BMAA) is a non-protein amino acid implicated in the neurodegenerative disease amyotrophic lateral sclerosis/Parkinson-dementia complex (ALS/PDC) on Guam. BMAA has recently been discovered in the brains of Alzheimer's patients in Canada and is produced by various species of cyanobacteria a...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/j.expneurol.2009.04.002
更新日期：2009-06-01 00:00:00
abstract：:Studies of the effects of lesions of the parietal cortex suggest that this region plays a critical role in visuomotor guidance, whereas experiments on the effects of disconnecting this region from visual input have been inconsistent in supporting this hypothesis. In the present experiment, monkeys with a unilateral oc...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1016/0014-4886(83)90161-9
更新日期：1983-07-01 00:00:00
abstract：:Five female adult Macaca nemestrina monkeys, given a unilateral intracarotid (ic) infusion of 2.3-3.5 mg of MPTP-HCl, were studied for 6-8 years. Two to 3 days after MPTP, the animals developed hemiparkinsonism characterized by rigidity and flexed posture of the arm contralateral to the side of infusion with episodes ...
journal_title：Experimental neurology
pub_type： 杂志文章
doi：10.1006/exnr.1998.6845
更新日期：1998-08-01 00:00:00
