Abstract:
:Disorders primarily affecting the nervous system comprise approximately one third of all established Mendelian genetic diseases in man. Recombinant DNA technology provides new approaches to the diagnosis and elucidation of the molecular pathology of these disorders. For a small but increasing number of disorders the DNA sequence coding for the involved protein has been used to define the precise molecular defect. An example is the Lesch-Nyhan syndrome. In many other situations, DNA fragments located near to the mutant gene can be used in family linkage studies to determine who is likely to have inherited the abnormal allele(s). Examples include Duchenne muscular dystrophy, Huntington's disease, and phenylketonuria. This technology offers unique opportunities to investigate the function of the nervous system in health and disease and will have a major impact on the neurosciences and the practice of clinical neurology.
journal_name
Dev Neuroscijournal_title
Developmental neuroscienceauthors
Hayden MR,Nichols JLdoi
10.1159/000112346subject
Has Abstractpub_date
1983-01-01 00:00:00pages
189-214issue
4-5eissn
0378-5866issn
1421-9859journal_volume
6pub_type
杂志文章,评审abstract::In order to investigate the possible influence of norepinephrine (NE) on neuronal development, the effects of neonatal NE depletion on postnatally developing cell populations were characterized in (1) the normal mouse cerebellum, and (2) a cerebellar model system with delayed germinal cell development produced by neon...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000112695
更新日期:1982-01-01 00:00:00
abstract::Fragile X syndrome (FXS) is a neurodevelopmental disorder characterized by intellectual disability, sensory hypersensitivity, and high incidences of autism spectrum disorders and epilepsy. These phenotypes are suggestive of defects in neural circuit development and imbalances in excitatory glutamatergic and inhibitory...
journal_title:Developmental neuroscience
pub_type: 杂志文章,评审
doi:10.1159/000329420
更新日期:2011-01-01 00:00:00
abstract::The auditory system of the cricket has the unusual ability to respond to deafferentation by compensatory growth and synapse formation. Auditory interneurons such as ascending neuron 2 (AN-2) in the cricket Gryllus bimaculatus possess a dendritic arbor that normally grows up to, but not over, the midline of the prothor...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000322887
更新日期:2011-01-01 00:00:00
abstract:BACKGROUND:Therapeutic hypothermia provides incomplete neuroprotection for neonatal hypoxic-ischemic encephalopathy (HIE). We examined whether hemodynamic goals that support autoregulation are associated with decreased brain injury and whether these relationships are affected by birth asphyxia or vary by anatomic regio...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000452833
更新日期:2017-01-01 00:00:00
abstract::In the developing brain the majority of neurons migrate from their birthplace to their final destination. This active movement is essential for the formation of cortical layers and nuclei. The impairment of migration does not affect the viability of neurons but often results in abnormal differentiation. The proper mig...
journal_title:Developmental neuroscience
pub_type: 杂志文章,评审
doi:10.1159/000109847
更新日期:2008-01-01 00:00:00
abstract::Proper eye formation depends on specific interactions between neural and ectodermal tissues coupled with temporally distinct gene expression and a regulated sequence of signaling events. The homeobox gene Pax6 is vitally important to the entire process of eye development in both vertebrates and invertebrates. Pax6 exp...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000082273
更新日期:2004-01-01 00:00:00
abstract::This study analyzed the role of dopamine D1 and D2 receptors in methylphenidate (MPH) conditioned place preference (CPP) in adolescent male and female rats, in addition to the role of these receptors in the effects of MPH on brain-derived neurotrophic factor (BDNF) in the dorsal striatum and nucleus accumbens. Using a...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000360636
更新日期:2014-01-01 00:00:00
abstract::In the era of therapeutic hypothermia, reliable preclinical studies are integral to successfully identify neuroprotective strategies to further improve outcomes of encephalopathy at term. We reviewed preclinical neuroprotection studies reported between January 2014 and June 2016 to assess the use of effective temperat...
journal_title:Developmental neuroscience
pub_type: 杂志文章,评审
doi:10.1159/000452859
更新日期:2017-01-01 00:00:00
abstract::Evidence from retroviral marking techniques and immortalized cell lines indicates that multipotential stem cells exist in many areas of the developing central nervous system. However, the factors that influence the commitment of these stem cells into distinct neuronal or glial lineages are not known. We have created a...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000111301
更新日期:1995-01-01 00:00:00
abstract::The neurochemical effect of postnatal exposure to methadone (METH), undernutrition (UND), and methadone and undernutrition (METH-UND) on the whole brain and cerebellum were examined in 21- and 60-day-old rats. At 21 days, body weight reductions of UND and METH-UND rats exceeded that of the METH animals. However, METH ...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000112698
更新日期:1982-01-01 00:00:00
abstract::Perinatal hypoxic ischemic (HI) brain injury is a leading cause of long-term neurological handicap in newborn babies. Recently, excessive activity of matrix metalloproteinases (MMPs), and in particular MMP-9, has been implicated in the aetiology of HI injuries to the immature brain. Our previous study suggested that M...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000343257
更新日期:2012-01-01 00:00:00
abstract::Neuronal heterotopia is a malformation of cortical development that is closely associated with epilepsy in humans. Despite emerging interest in the structure and function of the heterotopic cortex, little is known about the membrane properties and synaptic connections of these displaced neurons. We used whole-cell pat...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000017386
更新日期:1999-11-01 00:00:00
abstract::Rabbit central (CNS) and peripheral nervous system (PNS) myelin, as well as nonmyelinating pig oligodendrocytes in culture, were extracted at 0-4 degrees C with the nonionic detergent Triton X-114. The solubilized proteins were partitioned into the detergent-rich and detergent-depleted (aqueous) phases that form upon ...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000111935
更新日期:1989-01-01 00:00:00
abstract::Neuron-specific enolase (NSE) is a unique form of the glycolytic enzyme, enolase, which, in the brain, is strictly localized in differentiated neurons and neuroendocrine cells. In this report evidence is presented that NSE is first seen in the cell bodies of developing chick auditory and vestibular neurons shortly aft...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000112689
更新日期:1982-01-01 00:00:00
abstract::Excitotoxicity plays a key role during insults to the developing brain such as neonatal encephalopathy, stroke, and encephalopathy of prematurity. Such insults affect many thousands of infants each year. Excitotoxicity causes frank lesions due to cell death and gliosis and disturbs normal developmental process, leadin...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000464131
更新日期:2017-01-01 00:00:00
abstract::The developing retina is characterized by 'retinal waves', spontaneous depolarizations that propagate through a developing network of interneurons and retinal ganglion cells. Although the circuitry underlying retinal waves is well characterized, the secreted factors that are critical for its normal development are not...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000082283
更新日期:2004-01-01 00:00:00
abstract::Astrocytes in primary cultures constitute an exceedingly useful preparation for studies of astroglial development and function. These cells, however, demonstrate a pronounced plasticity in their reactions to culturing conditions. Thus, species and spatiotemporal region of CNS chosen for source of cells, dissociation p...
journal_title:Developmental neuroscience
pub_type: 杂志文章,评审
doi:10.1159/000112295
更新日期:1985-01-01 00:00:00
abstract::The postnatal development of the complexes of the electron transport chain in mitochondria isolated from rat brain synaptosomes was investigated. Synaptosomal brain mitochondria were isolated from rats aged 10-60 days, and the activities of mitochondrial complex I, complex II-III, complex IV and complex V were measure...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000111289
更新日期:1995-01-01 00:00:00
abstract::The myelin-associated glycoprotein (MAG) is a 100 K-dalton, integral membrane glycoprotein containing 30% carbohydrate that is in central and peripheral myelin sheaths. Recent research on its function during normal myelinogenesis and possible roles in the pathology of demyelinating diseases is reviewed. Emphasis is pl...
journal_title:Developmental neuroscience
pub_type: 杂志文章,评审
doi:10.1159/000112356
更新日期:1983-01-01 00:00:00
abstract::Perinatal asphyxia is a leading cause of brain injury in neonates, occurring in 2-4 per 1,000 live births, and there are limited treatment options. Because of their similarity to humans, nonhuman primates are ideal for performing preclinical tests of safety and efficacy for neurotherapeutic interventions. We previousl...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000327246
更新日期:2011-01-01 00:00:00
abstract::Choline acetyltransferase (ChAT), the biosynthetic enzyme for acetylcholine metabolism, is expressed in a human cholinergic neuroblastoma cell line; MC-IXC. We demonstrate that ChAT activity is regulated in this cell line by cell density. It is believed that the mechanism of stimulation of enzyme activity involves cel...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000111975
更新日期:1988-01-01 00:00:00
abstract::This study of the molecular forms of acetylcholinesterase in several rat brain regions using sucrose gradient centrifugation has revealed only two major forms throughout postnatal development: the 4S and 10S. In all regions studied (cerebral cortex, cerebellum, caudate nucleus, hippocampus, pons-medulla and superior c...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000112383
更新日期:1980-01-01 00:00:00
abstract::The norepinephrine content of hypothalamus in vehicle-treated control rats gradually increases between days 16 and 45 of life. Norepinephrine contents of the hypothalamus and mesencephalon of the thyroxine-treated (10 microgram/g b.w., s.c., days 8-10) animals also increase between days 16 and 21. Mesencephalic and hy...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000112377
更新日期:1980-01-01 00:00:00
abstract::Neurotransmitters play important roles in neurogenesis; in particular, acetylcholine and serotonin may regulate neurite elongation. Acetylcholine may also activate transcription factors such as early growth response protein 1 (EGR-1), which plays a role in neurite extension. N18TG2 neuroblastoma cells (which do not pr...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000354423
更新日期:2013-01-01 00:00:00
abstract::The long noncoding RNA MSNP1AS (moesin pseudogene 1, antisense) is a functional element that was previously associated with autism spectrum disorder (ASD) with genome-wide significance. Expression of MSNP1AS was increased 12-fold in the cerebral cortex of individuals with ASD and 22-fold in individuals with a genome-w...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000453258
更新日期:2016-01-01 00:00:00
abstract::Adult neuronal ceroid lipofuscinosis (NCL), also called Kufs' disease, is clinically distinct from the other NCLs. It is a rare condition which is difficult to diagnose. More than 50% of the reported cases of Kufs' disease are not adult NCL and correspond very likely to a heterogeneous spectrum of lipidoses. Various c...
journal_title:Developmental neuroscience
pub_type: 杂志文章,评审
doi:10.1159/000112182
更新日期:1991-01-01 00:00:00
abstract::Dissociated cells from chick embryo encephalon of 36 h of incubation have been cultivated on fibroblast, meningeal cell, or glial layers. Within the first 24 h of cultivation, cells adhered to the preformed layers more firmly than to plastic or collagen surfaces. After 48 h of cultivation, the brain cells cultured on ...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000112568
更新日期:1978-01-01 00:00:00
abstract::L-Ornithine decarboxylase (ODC), the rate-limiting enzyme of polyamine biosynthesis and thus a unique marker of the proliferation and maturation processes, has been localized by means of immunohistochemistry in the human central nervous system. The enzyme protein has not only been found in the developing nervous syste...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000111320
更新日期:1993-01-01 00:00:00
abstract::Parallel developmental studies of central nervous system myelin proteins and morphology (postnatal days 15-118; P15-118) confirm qualitative similarities but substantial quantitative differences between homozygous mld mice with Billings-Gagliardi and Wolf's 'USA' versus Matthieu's 'Swiss' genetic backgrounds. The USA ...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000112145
更新日期:1991-01-01 00:00:00
abstract::The proximodistal axonal transport of choline acetyltransferase (ChAc) has been studied in the chick sciatic nerve in the absence of any experimental manipulation. The phenomenon utilized by us is a physiological transient fall in the activity of ChAc. This fall occurs in most areas of the chick central nervous system...
journal_title:Developmental neuroscience
pub_type: 杂志文章
doi:10.1159/000112484
更新日期:1979-01-01 00:00:00