Abstract：
:Spinocerebellar ataxia type 1 (SCA1) is a devastating neurodegenerative disorder in which an abnormally expanded polyglutamine tract is inserted into causative ataxin-1 proteins. We have previously shown that SCA1 knockin (SCA1-KI) mice over 6 months of age exhibit a degeneration of motor neuron axons and their encasing myelin sheaths, as reported in SCA1 patients. We examined whether axon degeneration precedes myelin degeneration or vice versa in SCA1-KI mice and then attempted to mitigate motor neuron degeneration by intrathecally administering mesenchymal stem cells (MSCs). Temporal examination of the diameters of motor neuron axons and their myelin sheaths revealed a decrease in diameter of the axon but not of the myelin sheaths in SCA1-KI mice as early as 1 month of age, which suggests secondary degeneration of the myelin sheaths. We injected MSCs into the intrathecal space of SCA1-KI mice at 1 month of age, which resulted in a significant suppression of degeneration of both motor neuron axons and myelin sheaths, even 6 months after the MSC injection. Thus, MSCs effectively suppressed peripheral nervous system degeneration in SCA1-KI mice. It has not yet been clarified how clinically administered MSCs exhibit significant therapeutic effects in patients with SCA1. The morphological evidence presented in this current mouse study might explain the mechanisms that underlie the therapeutic effects of MSCs that are observed in patients with SCA1.
journal_name
J Neurosci Resjournal_title
Journal of neuroscience researchauthors
Mieda T,Suto N,Iizuka A,Matsuura S,Iizuka H,Takagishi K,Nakamura K,Hirai Hdoi
10.1002/jnr.23698subject
Has Abstractpub_date
2016-03-01 00:00:00pages
246-52issue
3eissn
0360-4012issn
1097-4547journal_volume
94pub_type
杂志文章abstract：:Antibody-producing hybridoma cells specifically bind to microgram quantities of antigen molecules adsorbed onto the surface of plastic microtiter plates. The binding of hybridoma cells to nonantigen is optimally below 5%, similar binding of non-antibody-producing cells is 4-7%, compared to the binding of the hybridoma...
journal_title：Journal of neuroscience research
pub_type： 杂志文章
doi：10.1002/jnr.490150312
更新日期：1986-01-01 00:00:00
abstract：:Traumatic injury to the adult central nervous system (CNS) results in a rapid response from resident astrocytes, a process often referred to as reactive astrogliosis or glial scarring. The robust formation of the glial scar and its associated extracellular matrix (ECM) molecules have been suggested to interfere with a...
journal_title：Journal of neuroscience research
pub_type： 杂志文章,评审
doi：10.1002/1097-4547(20010115)63:2<109::AID-JNR1002>3
更新日期：2001-01-15 00:00:00
abstract：:We review three principally different forms of injury-induced synaptic alterations. (1) Displacement of presynaptic terminals from perikarya and dendrites of axotomized neurons, (2) central changes in primary afferent terminals of peripherally axotomized sensory ganglion cells, and (3) anterograde Wallerian-type degen...
journal_title：Journal of neuroscience research
pub_type： 杂志文章,评审
doi：
更新日期：1999-10-01 00:00:00
abstract：:A series of AMPA [(R,S)-alpha-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid] analogues were evaluated for activity at homomeric, heteromeric, and chimeric rat GluR1(o) and GluR3(o) receptors expressed in Xenopus oocytes, using the two-electrode voltage clamp technique. The formation of heteromeric receptor compl...
journal_title：Journal of neuroscience research
pub_type： 杂志文章
doi：10.1002/(sici)1097-4547(19970715)49:2<176::aid-jnr
更新日期：1997-07-15 00:00:00
abstract：:Several recently discovered lines of evidence support the involvement of myelin basic protein (BP)-specific T cells in multiple sclerosis (MS). To identify potentially relevant immunodominant T cell epitopes, human BP (Hu-BP)-reactive T cell lines were selected from MS and normal donors and tested for reactivity to cl...
journal_title：Journal of neuroscience research
pub_type： 杂志文章
doi：10.1002/jnr.490280215
更新日期：1991-02-01 00:00:00
abstract：:The present experiments tested the hypothesis that one of the critical mechanisms underlying genetically defined aggressiveness involves brain serotonin 5-HT1A receptors. 5-HT1A receptor density, the receptor mRNA expression in brain structures, and functional correlates for 5-HT1A receptors identified as 8-OH-DPAT-in...
journal_title：Journal of neuroscience research
pub_type： 杂志文章
doi：10.1002/jnr.20456
更新日期：2005-04-15 00:00:00
abstract：:The exposure of SY5Y neuroblastoma cells to high concentrations of glucose, fructose, or galactose is an experimental model commonly used for in vitro evaluation of typical neuronal alterations observed in diabetes mellitus. In the present study, we observed that 2 weeks of exposure to high carbohydrate concentrations...
journal_title：Journal of neuroscience research
pub_type： 杂志文章
doi：
更新日期：1999-09-01 00:00:00
abstract：:Accumulating evidence indicates an immunosuppressive role for CD4(+)CD25(+) regulatory T cells (Tregs) in autoimmune diseases. Although an impaired Treg function in patients with relapsing-remitting multiple sclerosis (RR-MS) has been reported recently, no information is available so far about Treg function in the pro...
journal_title：Journal of neuroscience research
pub_type： 杂志文章
doi：10.1002/jnr.20852
更新日期：2006-06-01 00:00:00
abstract：:It was previously reported that a monoclonal antibody to beta-amyloid precursor protein (mab22C11; Boehringer Mannheim, Indianapolis, IN) labels an intermediate filament-associated protein (beta APP-IFAP) in cultured human skin fibroblasts (Dooley et al.: J Neurosci Res 33:60-67, 1992). The time course of its expressi...
journal_title：Journal of neuroscience research
pub_type： 杂志文章
doi：10.1002/jnr.490380605
更新日期：1994-08-15 00:00:00
abstract：:Nascent neural crest cells derived from explanted E12 embryonic rat caudal neural tubes were used as an assay system to investigate the effects of fibroblast growth factors on neural crest cell (NCC) survival, proliferation, migration, and differentiation. In vitro and in vivo all NCC express low affinity nerve growth...
journal_title：Journal of neuroscience research
pub_type： 杂志文章
doi：10.1002/jnr.490360106
更新日期：1993-09-01 00:00:00
abstract：:Ex vivo gene therapy is emerging as a promising approach for the treatment of neurodegenerative diseases and central nervous system (CNS) trauma. We have shown previously that human adult astrocytes can be expanded in vitro and can express various therapeutic transgenes (Ridet et al. [1999] Hum. Gene Ther. 10:271-280;...
journal_title：Journal of neuroscience research
pub_type： 杂志文章
doi：10.1002/jnr.10617
更新日期：2003-06-15 00:00:00
abstract：:Primary fibroblasts initiated from skin biopsies of Wistar rats were transfected with a plasmid that encodes the human growth hormone and the neomycin resistance genes. Cell clones selected for G418 resistance and expressing high levels of human growth hormone were propagated in vitro and subsequently transplanted int...
journal_title：Journal of neuroscience research
pub_type： 杂志文章
doi：10.1002/jnr.490290304
更新日期：1991-07-01 00:00:00
abstract：:Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease. Progressive and systemic loss of motor neurons with gliosis in the central nervous system (CNS) is a neuropathological hallmark of ALS. Chondroitin sulfate proteoglycans (CSPGs) are the major components of the extracellular matrix of the mammali...
journal_title：Journal of neuroscience research
pub_type： 杂志文章
doi：10.1002/jnr.24127
更新日期：2018-02-01 00:00:00
abstract：:There are a considerable number of studies concerning the behavioral effects of physical exercise on the epileptic brain; however, the intracellular signaling mechanisms involved remain unclear. We investigated the effects of aerobic exercise on hippocampal levels of brain-derived neurotrophic factor (BDNF), expressio...
journal_title：Journal of neuroscience research
pub_type： 杂志文章
doi：10.1002/jnr.24196
更新日期：2018-05-01 00:00:00
abstract：:The blood-brain barrier (BBB) refers to the network of microvessels that selectively restricts the passage of substances between the circulation and the central nervous system (CNS). This microvascular network is comprised of arterioles, capillaries and venules, yet the respective contribution of each of these to the ...
journal_title：Journal of neuroscience research
pub_type： 杂志文章
doi：10.1002/jnr.22316
更新日期：2010-05-15 00:00:00
abstract：:Peroxisome proliferator-activated receptors (PPARs) are ligand-activated transcription factors that form a subfamily within the steroid hormone receptor group. Recent work has shown that one member of this group, PPARgamma, plays a central role in adipocyte differentiation. As oligodendrocytes are major lipid-producin...
journal_title：Journal of neuroscience research
pub_type： 杂志文章
doi：10.1002/(SICI)1097-4547(19980301)51:5<563::AID-JNR
更新日期：1998-03-01 00:00:00
abstract：:Although upregulation of ryanodine receptor (RyR)-1 and -2 is mediated through the activation of dopamine D1 receptors (D1DRs) in the development of psychostimulant-induced place preference, little is known about how such increased expressions of RyRs are negatively regulated. This study investigated negative regulato...
journal_title：Journal of neuroscience research
pub_type： 杂志文章
doi：10.1002/jnr.23058
更新日期：2012-08-01 00:00:00
abstract：:Brain-derived neurotrophic factor (BDNF) acutely modulates the efficacy of central glutamatergic synapses via activation of the receptor tyrosine kinase TrkB. On a longer time scale, recent evidence suggests an additional role of TrkB signaling in the formation of excitatory synaptic connections. Here, we have overexp...
journal_title：Journal of neuroscience research
pub_type： 杂志文章
doi：10.1002/jnr.10007
更新日期：2001-11-01 00:00:00
abstract：:The Shaw subfamily of potassium channel genes, including Kv3.1, are highly expressed within the auditory nuclei of the brainstem, where they have been implicated in the characteristic response properties of particular types of neurons. Potassium currents carried by Kv3.1 are voltage-dependent, have a high activation t...
journal_title：Journal of neuroscience research
pub_type： 杂志文章
doi：10.1002/(sici)1097-4547(19991215)58:6<791::aid-jnr
更新日期：1999-12-15 00:00:00
abstract：:We report a study of the characterization of voltage-activated potassium (K+) currents in retinal ON bipolar cells in zebrafish. At single-channels levels, the open probability of the K+ channels increased when the membrane potential was increased. The maximal open proportion was 0.76+/-0.05 under our testing conditio...
journal_title：Journal of neuroscience research
pub_type： 杂志文章
doi：10.1002/jnr.20637
更新日期：2005-11-01 00:00:00
abstract：:Essential to understanding the roles proteins and structural elements play at the synapse is to understand the development, remodeling and reinnervation of peripheral neuromuscular junctions. It has, however, been a challenging task to label and visualize neuromuscular junctions. In this paper we demonstrate how adeno...
journal_title：Journal of neuroscience research
pub_type： 杂志文章
doi：10.1002/1097-4547(20000701)61:1<61::AID-JNR7>3.0.C
更新日期：2000-07-01 00:00:00
abstract：:Alpha2-adrenergic receptors control norepinephrine (NE) release and tumor necrosis factor-alpha (TNF) production from neurons. TNF regulates NE release, depending on alpha2-adrenergic receptor functioning. The relationship between TNF production in the brain and alpha2-adrenergic receptor activation could have profoun...
journal_title：Journal of neuroscience research
pub_type： 杂志文章
doi：10.1002/jnr.20407
更新日期：2005-03-15 00:00:00
abstract：:Although the thalamus is not considered primarily as a limbic structure, abundant evidence indicates the essential role of the thalamus as a modulator of limbic functions indirectly through the amygdala. The amygdala is a central component of the limbic system and serves an essential role in modulating the core proces...
journal_title：Journal of neuroscience research
pub_type： 杂志文章
doi：10.1002/jnr.24235
更新日期：2018-07-01 00:00:00
abstract：:Contacts between horizontal and bipolar cells are described in the retina of the teleost Eugerres plumieri. A single, long expansion observed in the external cone horizontal cells makes contact by means of a terminal button with the cell body of a bipolar. It represents the only contact between this class of horizonta...
journal_title：Journal of neuroscience research
pub_type： 杂志文章
doi：10.1002/jnr.490060111
更新日期：1981-01-01 00:00:00
abstract：:The presence of acetylcholinesterase has been detected in the thymus of several species both biochemically and histochemically. In this study we have investigated the molecular forms and the level of this enzyme in separate compartments of the murine thymus and in different thymocyte subpopulations. Similar levels of ...
journal_title：Journal of neuroscience research
pub_type： 杂志文章
doi：10.1002/jnr.490290210
更新日期：1991-06-01 00:00:00
abstract：:In searching for possible differences in the composition of acetylcholinesterase (AChE) and butyrylcholinesterase (BuChE) forms in dystrophic brain, the distribution of various enzyme molecules in normal (NB) and dystrophic (DB) 129B6F1/J mouse brain has been investigated. The tissue was sequentially extracted with sa...
journal_title：Journal of neuroscience research
pub_type： 杂志文章
doi：10.1002/(SICI)1097-4547(19960115)43:2<224::AID-JNR
更新日期：1996-01-15 00:00:00
abstract：:The midbrain central gray (periaqueductal gray; PAG) mediates defensive behaviors and is implicated in the rewarding effects of opiate drugs. Projections from the PAG to the ventral tegmental area (VTA) suggest that this region might also regulate behaviors involving motivation and cognition. However, studies have not...
journal_title：Journal of neuroscience research
pub_type： 杂志文章
doi：10.1002/jnr.22265
更新日期：2010-04-01 00:00:00
abstract：:High levels of the proto-oncogene product, pp60c-src, have been found in developing and adult neural tissues as well as in certain fully mature cells of the hematopoietic lineage, e.g., platelets and myelomonocytes. Adrenal medullary chromaffin cells exhibit characteristics of both types of cells, i.e., they are deriv...
journal_title：Journal of neuroscience research
pub_type： 杂志文章
doi：10.1002/jnr.490240107
更新日期：1989-09-01 00:00:00
abstract：:An aminopeptidase purified to homogeneity from cytosol of rat brain cleaved dynorphins having 5-17 residues and selected proenkephalins at the Tyr-Gly bond only to release Tyr and the desTyr fragments. The enzyme protein consisted of a single polypeptide chain of Mr 103,000 and was inhibited by puromycin, bestatin, an...
journal_title：Journal of neuroscience research
pub_type： 杂志文章
doi：10.1002/jnr.490110311
更新日期：1984-01-01 00:00:00
abstract：:Ten days after bilateral injections of ibotenic acid into the nucleus basalis, rats injected daily (i.m.) with ganglioside GM1 were protected from anterograde degeneration of cholinergic projections to the fronto-lateral cortex. This protection was reflected by reduced losses (associated with ibotenic acid lesions) of...
journal_title：Journal of neuroscience research
pub_type： 杂志文章
doi：10.1002/jnr.490200411
更新日期：1988-08-01 00:00:00
