The "campus syndrome" in pigs: neurological, neurophysiological, and neuropharmacological characterization of a new genetic animal model of high-frequency tremor.

Abstract:

:Inherited neurological diseases in animals are of interest to a wide range of scientific disciplines, particularly because such animals may be suited as genetic animal models for respective human disorders. Because the pig has a number of anatomic and physiologic features similar to those of human beings, this species is becoming increasingly popular in biomedical research. The usefulness of pigs as genetic models of neurological diseases is illustrated by the porcine model of malignant hyperthermia (MH), i.e., a frequently fatal myopathic disease in both pigs and humans. In the present study, we describe a new hereditary movement disorder in Pietrain pigs, which may represent a useful genetic animal model of high-frequency tremor. Because the disorder was first detected in the offspring of a boar named "Campus," we use the term "Campus syndrome" in this respect. Segregation analysis of breeding studies indicates that the syndrome is inherited as a monogenic dominant trait. DNA-based testing of the mutation involved in MH myopathy showed that expression of the Campus syndrome in pigs is not dependent on homozygosity for the MH mutation. In affected pigs, the Campus syndrome develops at an average age of 27 days. The syndrome is characterized by muscular weakness and a very intense tremor of the legs when standing and walking but not when at rest in a lying position. The intensity of tremor and muscular weakness progressively increases with age, resulting in pronounced postural instability. Despite these neurological abnormalities, body weight gain in affected pigs does not differ from that in unaffected siblings.(ABSTRACT TRUNCATED AT 250 WORDS)

journal_name

Exp Neurol

journal_title

Experimental neurology

authors

Richter A,Wissel J,Harlizius B,Simon D,Schelosky L,Scholz U,Poewe W,Löscher W

doi

10.1006/exnr.1995.1050

subject

Has Abstract

pub_date

1995-08-01 00:00:00

pages

205-13

issue

2

eissn

0014-4886

issn

1090-2430

pii

S0014-4886(85)71050-3

journal_volume

134

pub_type

杂志文章
  • The mode of axonal regeneration of locus coeruleus neurons in the lateral geniculate nucleus following neonatal 6-hydroxydopamine treatment.

    abstract::The effects of neonatal 6-hydroxydopamine (6-OHDA) treatment on the projection of locus coeruleus (LC) neurons to the dorsal part of the lateral geniculate nucleus (dLGN) were examined using histofluorescence and electrophysiological methods. Histofluorescence studies confirmed the previous results of Gustafson and Mo...

    journal_title:Experimental neurology

    pub_type: 杂志文章

    doi:10.1016/0014-4886(91)90128-y

    authors: Aoki F,Nakamura S

    更新日期:1991-06-01 00:00:00

  • Evidence of axon connectivity across a spinal cord transection in rats treated with epidural stimulation and motor training combined with olfactory ensheathing cell transplantation.

    abstract::Olfactory ensheathing cells (OECs) are unique glia that support axon outgrowth in the olfactory system, and when used as cellular therapy after spinal cord injury, improve recovery and axon regeneration. Here we assessed the effects of combining OEC transplantation with another promising therapy, epidural electrical s...

    journal_title:Experimental neurology

    pub_type: 杂志文章

    doi:10.1016/j.expneurol.2018.07.015

    authors: Thornton MA,Mehta MD,Morad TT,Ingraham KL,Khankan RR,Griffis KG,Yeung AK,Zhong H,Roy RR,Edgerton VR,Phelps PE

    更新日期:2018-11-01 00:00:00

  • Endothelial cell loss is not a major cause of neuronal and glial cell death following contusion injury of the spinal cord.

    abstract::Contusion of the spinal cord causes an immediate local loss of neurons and disruption of vasculature; additional loss continues thereafter. To explore the possibility of a causal link between delayed endothelial cell (EC) death and secondary neural cell loss, we evaluated neural and endothelial cell survival, and meas...

    journal_title:Experimental neurology

    pub_type: 杂志文章

    doi:10.1016/j.expneurol.2006.05.028

    authors: Casella GT,Bunge MB,Wood PM

    更新日期:2006-11-01 00:00:00

  • Expression of herpes simplex virus type 2 protein ICP10 PK rescues neurons from apoptosis due to serum deprivation or genetic defects.

    abstract::Previous studies have shown that the herpes simplex virus type 2 protein kinase ICP10 PK activates the Ras/MEK/MAPK pathway in nonneuronal cells. Here we report that ectopically expressed ICP10 PK has anti-apoptotic activity in various paradigms of neuronal cell death. Neuronally differentiated PC12 cells and primary ...

    journal_title:Experimental neurology

    pub_type: 杂志文章

    doi:10.1006/exnr.2001.7849

    authors: Perkins D,Yu Y,Bambrick LL,Yarowsky PJ,Aurelian L

    更新日期:2002-03-01 00:00:00

  • A histochemical study of lateral longissimus muscle in rat.

    abstract::Rat axial muscle previously has not been studied histochemically. We were interested in determining the fiber composition and fiber distribution in rat lateral longissimus (LL), the large epaxial dorsiflexor muscle active during sexual posturing in the female rat and to determine if estrogen replacement in ovariectomi...

    journal_title:Experimental neurology

    pub_type: 杂志文章

    doi:10.1016/0014-4886(83)90229-7

    authors: Schwartz-Giblin S,Rosello L,Pfaff DW

    更新日期:1983-02-01 00:00:00

  • Minocycline delays disease onset and mortality in reovirus encephalitis.

    abstract::Minocycline is neuroprotective in many experimental models of neurodegenerative diseases and central nervous system (CNS) injury but has not previously been tested in a model of viral encephalitis. Experimental infection of neonatal mice with neurotropic reoviruses is a classic model for studying the pathogenesis of v...

    journal_title:Experimental neurology

    pub_type: 杂志文章

    doi:10.1016/j.expneurol.2004.11.015

    authors: Richardson-Burns SM,Tyler KL

    更新日期:2005-04-01 00:00:00

  • The role of growth factors as a therapeutic approach to demyelinating disease.

    abstract::A variety of growth factors are being explored as therapeutic agents relevant to the axonal and oligodendroglial deficits that occur as a result of demyelinating lesions such as are evident in Multiple Sclerosis (MS). This review focuses on five such proteins that are present in the lesion site and impact oligodendroc...

    journal_title:Experimental neurology

    pub_type: 杂志文章,评审

    doi:10.1016/j.expneurol.2016.02.023

    authors: Huang Y,Dreyfus CF

    更新日期:2016-09-01 00:00:00

  • Antagonizing bone morphogenetic protein 4 attenuates disease progression in a rat model of amyotrophic lateral sclerosis.

    abstract::Amyotrophic lateral sclerosis (ALS) is an adult-onset, fatal neurodegenerative syndrome characterized by the systemic loss of motor neurons with prominent astrocytosis and microgliosis in the spinal cord and brain. Astrocytes play an essential role in maintaining extracellular microenvironments that surround motor neu...

    journal_title:Experimental neurology

    pub_type: 杂志文章

    doi:10.1016/j.expneurol.2018.06.009

    authors: Shijo T,Warita H,Suzuki N,Ikeda K,Mitsuzawa S,Akiyama T,Ono H,Nishiyama A,Izumi R,Kitajima Y,Aoki M

    更新日期:2018-09-01 00:00:00

  • Localization of alpha-, beta-, and gamma-synuclein during neuronal development and alterations associated with the neuronal response to axonal trauma.

    abstract::Genetic and protein studies have indicated abnormalities in alpha-synuclein in neurodegenerative diseases. However, the developmental localization and cellular role of synuclein isoforms is contentious. We investigated the cellular localization of alpha-, beta-, and gamma-synuclein in developing cultured rat neurons a...

    journal_title:Experimental neurology

    pub_type: 杂志文章

    doi:10.1016/s0014-4886(03)00108-0

    authors: Quilty MC,Gai WP,Pountney DL,West AK,Vickers JC

    更新日期:2003-07-01 00:00:00

  • Functional MRI at 4.7 tesla of the rat brain during electric stimulation of forepaw, hindpaw, or tail in single- and multislice experiments.

    abstract::Stimulation of peripheral nerves activates corresponding regions in sensorimotor cortex. We have applied functional magnetic resonance imaging (fMRI) techniques to monitor activated brain regions by means of measuring changes of blood oxygenation level-dependent contrast during electric stimulation of the forepaw, hin...

    journal_title:Experimental neurology

    pub_type: 杂志文章

    doi:10.1006/exnr.2000.7524

    authors: Spenger C,Josephson A,Klason T,Hoehn M,Schwindt W,Ingvar M,Olson L

    更新日期:2000-12-01 00:00:00

  • NGF released from a polymer matrix prevents loss of ChAT expression in basal forebrain neurons following a fimbria-fornix lesion.

    abstract::Following a unilateral fimbria-fornix lesion, the delivery of nerve growth factor (NGF) to the ipsilateral lateral ventricle of the rat can prevent the lesion-induced loss of choline acetyltransferase (ChAT) expression in the ipsilateral medial septum and vertical diagonal band region. In the present study, the abilit...

    journal_title:Experimental neurology

    pub_type: 杂志文章

    doi:10.1016/0014-4886(90)90049-x

    authors: Hoffman D,Wahlberg L,Aebischer P

    更新日期:1990-10-01 00:00:00

  • Increased alpha-synuclein aggregation following limited cleavage by certain matrix metalloproteinases.

    abstract::Recent evidence indicates that protein aggregation and in particular the formation of toxic protein oligomers is a key mechanism in synucleinopathies such as Parkinson's disease (PD). Post mortem brain tissue studies as well as animal studies furthermore suggest that matrix metalloproteinases (MMPs) are also involved ...

    journal_title:Experimental neurology

    pub_type: 杂志文章

    doi:10.1016/j.expneurol.2008.10.010

    authors: Levin J,Giese A,Boetzel K,Israel L,Högen T,Nübling G,Kretzschmar H,Lorenzl S

    更新日期:2009-01-01 00:00:00

  • Intraspinal microstimulation for respiratory muscle activation.

    abstract::A complex propriospinal network is synaptically coupled to phrenic and intercostal motoneurons, and this makes it difficult to predict how gray matter intraspinal microstimulation (ISMS) will recruit respiratory motor units. We therefore mapped the cervical and high thoracic gray matter at locations which ISMS activat...

    journal_title:Experimental neurology

    pub_type: 杂志文章

    doi:10.1016/j.expneurol.2017.12.014

    authors: Sunshine MD,Ganji CN,Reier PJ,Fuller DD,Moritz CT

    更新日期:2018-04-01 00:00:00

  • Neuronal migration on laminin involves neuronal contact formation followed by nuclear movement inside a preformed process.

    abstract::Neuronal migration was investigated in rodent cerebellum in vitro and in vivo. Time-lapse video recording showed that cultured neurons migrated on laminin by first extending neurites that formed contacts with other neurons. This was followed by movement of the cell nucleus inside the preformed process. No guidance cue...

    journal_title:Experimental neurology

    pub_type: 杂志文章

    doi:10.1016/0014-4886(92)90119-b

    authors: Liesi P

    更新日期:1992-08-01 00:00:00

  • Prenatal exposure to alcohol alters short-term plasticity in hippocampus.

    abstract::Paired-pulse potentiation was studied in the CA1 region of rats exposed prenatally to alcohol. Pregnant rats were fed a liquid diet containing 35% ethanol-derived calories on days 3 to 21 of gestation. Control animals were fed liquid diets without ethanol. When the pups of both groups were 40 to 60 days old, hippocamp...

    journal_title:Experimental neurology

    pub_type: 杂志文章

    doi:10.1016/0014-4886(86)90203-7

    authors: Hablitz JJ

    更新日期:1986-08-01 00:00:00

  • The angiotensin converting enzyme inhibitor captopril protects nigrostriatal dopamine neurons in animal models of parkinsonism.

    abstract::Parkinson's disease (PD) is a progressive neurodegenerative disorder characterized by a prominent loss of nigrostriatal dopamine (DA) neurons with an accompanying neuroinflammation. The peptide angiotensin II (AngII) plays a role in oxidative-stress induced disorders and is thought to mediate its detrimental actions v...

    journal_title:Experimental neurology

    pub_type: 杂志文章

    doi:10.1016/j.expneurol.2013.10.014

    authors: Sonsalla PK,Coleman C,Wong LY,Harris SL,Richardson JR,Gadad BS,Li W,German DC

    更新日期:2013-12-01 00:00:00

  • Neurogenesis in postnatal mouse dorsal root ganglia.

    abstract::Neurogenesis continues in various regions of the central nervous system (CNS) throughout life. As the mitogen basic fibroblast growth factor (bFGF) can proliferate neuronal precursors of CNS neurons in culture, and is also upregulated within adult dorsal root ganglia following axotomy, it is possible that the postnata...

    journal_title:Experimental neurology

    pub_type: 杂志文章

    doi:10.1006/exnr.2001.7761

    authors: Namaka MP,Sawchuk M,MacDonald SC,Jordan LM,Hochman S

    更新日期:2001-11-01 00:00:00

  • Opposing effects by pituitary adenylate cyclase-activating polypeptide and vasoactive intestinal peptide on hippocampal synaptic transmission.

    abstract::Pituitary adenylate cyclase-activating polypeptide (PACAP), vasoactive intestinal peptide (VIP), and their receptors have been localized within the hippocampus but their physiological function on synaptic transmission is still unclear. We investigated the effects of PACAP and VIP on evoked excitatory postsynaptic curr...

    journal_title:Experimental neurology

    pub_type: 杂志文章

    doi:10.1016/S0014-4886(03)00300-5

    authors: Ciranna L,Cavallaro S

    更新日期:2003-12-01 00:00:00

  • Female rats are more sensitive to the locomotor alterations following quinolinic acid-induced striatal lesions: effects of striatal transplants.

    abstract::The effects of sex differences and hormonal factors on the locomotor alterations following intrastriatal injections of quinolinic acid (QA) and the ability of fetal striatal transplants to reverse those effects were examined. Male, female, or ovariectomized female rats received bilateral injections of 150 nmol QA or v...

    journal_title:Experimental neurology

    pub_type: 杂志文章

    doi:10.1016/0014-4886(91)90105-l

    authors: Emerich DF,Zubricki EM,Shipley MT,Norman AB,Sanberg PR

    更新日期:1991-03-01 00:00:00

  • Low-doses of cisplatin injure hippocampal synapses: a mechanism for 'chemo' brain?

    abstract::Chemotherapy-related cognitive deficits are a major neurological problem, but the underlying mechanisms are unclear. The death of neural stem/precursor cell (NSC) by cisplatin has been reported as a potential cause, but this requires high doses of chemotherapeutic agents. Cisplatin is frequently used in modern oncolog...

    journal_title:Experimental neurology

    pub_type: 杂志文章

    doi:10.1016/j.expneurol.2014.02.020

    authors: Andres AL,Gong X,Di K,Bota DA

    更新日期:2014-05-01 00:00:00

  • Matrices, scaffolds, and carriers for protein and molecule delivery in peripheral nerve regeneration.

    abstract::Local application of exogenous agents with neurotrophic properties enhances the regenerative capacity of injured neurons, especially following reconstructions of long nerve gaps and delayed nerve repairs. Recent advances in biomaterials and biomedical engineering have provided options for the sustained and controlled ...

    journal_title:Experimental neurology

    pub_type: 杂志文章,评审

    doi:10.1016/j.expneurol.2018.08.014

    authors: Tajdaran K,Chan K,Gordon T,Borschel GH

    更新日期:2019-09-01 00:00:00

  • Stimulation of the subthalamic region at 20 Hz slows the development of grip force in Parkinson's disease.

    abstract::Excessive synchronization of basal ganglia neuronal activity at ~20 Hz is characteristic of patients with untreated Parkinson's disease (PD). Correlative evidence suggests that this activity may contribute to bradykinesia. Attempts to demonstrate causality through stimulation imposed synchronization at 20 Hz in the re...

    journal_title:Experimental neurology

    pub_type: 杂志文章

    doi:10.1016/j.expneurol.2011.05.018

    authors: Chen CC,Lin WY,Chan HL,Hsu YT,Tu PH,Lee ST,Chiou SM,Tsai CH,Lu CS,Brown P

    更新日期:2011-09-01 00:00:00

  • Unity of costal and crural diaphragmatic activity in respiration.

    abstract::In chronically implanted rats, we examined the respiratory EMG activity of the two parts of the diaphragm, costal and crural, during sleep and wakefulness. Their activity was compared and contrasted with that of the EMG activity of the cricothyroid muscle. Whether in wakefulness, while grooming and drinking, or in non...

    journal_title:Experimental neurology

    pub_type: 杂志文章

    doi:10.1016/0014-4886(85)90051-2

    authors: Pollard MJ,Megirian D,Sherrey JH

    更新日期:1985-10-01 00:00:00

  • Anticonvulsant actions of MK-801 on the lithium-pilocarpine model of status epilepticus in rats.

    abstract::MK-801, a noncompetitive N-methyl-D-aspartate (NMDA) receptor antagonist, was tested for anticonvulsant effects in rats using two seizure models, coadministration of lithium and pilocarpine and administration of a high dose of pilocarpine alone. Three major results are reported. First, pretreatment with MK-801 produce...

    journal_title:Experimental neurology

    pub_type: 杂志文章

    doi:10.1016/0014-4886(89)90091-5

    authors: Ormandy GC,Jope RS,Snead OC 3rd

    更新日期:1989-11-01 00:00:00

  • Targeted overexpression of human alpha-synuclein in oligodendroglia induces lesions linked to MSA-like progressive autonomic failure.

    abstract::Multiple system atrophy (MSA) is a rare neurodegenerative disease of undetermined cause manifesting with progressive autonomic failure (AF), cerebellar ataxia and parkinsonism due to neuronal loss in multiple brain areas associated with (oligodendro)glial cytoplasmic alpha-synuclein (alpha SYN) inclusions (GCIs). Usin...

    journal_title:Experimental neurology

    pub_type: 杂志文章

    doi:10.1016/j.expneurol.2010.05.008

    authors: Stemberger S,Poewe W,Wenning GK,Stefanova N

    更新日期:2010-08-01 00:00:00

  • Ghrelin prevents 1-methyl-4-phenylpyridinium ion-induced cytotoxicity through antioxidation and NF-kappaB modulation in MES23.5 cells.

    abstract::Ghrelin, a 28-amino acid peptide, is an endogenous ligand for the growth hormone secretagogue (GHS) receptor. Our previous data showed that ghrelin could inhibit apoptosis in Parkinson's disease (PD) models both in vitro and in vivo. There is now growing evidence that oxidative stress has a critical role in the etiolo...

    journal_title:Experimental neurology

    pub_type: 杂志文章

    doi:10.1016/j.expneurol.2009.11.009

    authors: Liu L,Xu H,Jiang H,Wang J,Song N,Xie J

    更新日期:2010-03-01 00:00:00

  • Transient damage to the axonal transport system without Wallerian degeneration by acute nerve compression.

    abstract::The aim of this study was to examine whether acute nerve compression damages an axonal transport system based on microtubules and how the fibers recover after the compression. A 5-mm segment of the tibial nerve of male wistar rat was compressed with a specially designed clip. Functional recovery was assessed using Tib...

    journal_title:Experimental neurology

    pub_type: 杂志文章

    doi:10.1006/exnr.1997.6601

    authors: Kitao A,Hirata H,Morita A,Yoshida T,Uchida A

    更新日期:1997-10-01 00:00:00

  • Periaqueductal gray inhibition of trigeminal subnucleus caudalis unitary responses evoked by dentine and nonnoxious facial stimulation.

    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

    authors: Figueiras R,Buño W Jr,García-Austt E,Delgado JM

    更新日期:1983-07-01 00:00:00

  • Injury-induced class 3 semaphorin expression in the rat spinal cord.

    abstract::In this study we evaluate the expression of all members of the class 3 semaphorins and their receptor components following complete transection and contusion lesions of the adult rat spinal cord. Following both types of lesions the expression of all class 3 semaphorins is induced in fibroblast in the neural scar. The ...

    journal_title:Experimental neurology

    pub_type: 杂志文章

    doi:10.1006/exnr.2002.7884

    authors: De Winter F,Oudega M,Lankhorst AJ,Hamers FP,Blits B,Ruitenberg MJ,Pasterkamp RJ,Gispen WH,Verhaagen J

    更新日期:2002-05-01 00:00:00

  • Interaction of norepinephrine and superior cervical ganglion input in the rat pineal body.

    abstract::The effects of superior cervical ganglion stimulation and the local application of norepinephrine or its antagonist propranolol were studied in 18 pineal cells. In 61% of the pineal cells, stimulation elicited excitation; the same cells also responded by excitation to norepinephrine ejection. When stimulation and nore...

    journal_title:Experimental neurology

    pub_type: 杂志文章

    doi:10.1016/0014-4886(85)90149-9

    authors: Reyes-Vazquez C,Dafny N

    更新日期:1985-12-01 00:00:00