当前位置： SCI文献检索 > JOURNAL OF THE NEUROLOGICAL SCIENCES期刊下所有文献 > Regional distribution of glycoasparagine storage material in the brain in aspartylglycosaminuria.

Regional distribution of glycoasparagine storage material in the brain in aspartylglycosaminuria.

Abstract：

:We have studied the regional distribution of glycoasparagine storage material in the brain in aspartylglycosaminuria, a condition characterized by inherited deficiency of lysosomal N-aspartyl-beta-N-acetylglucosamine amidohydrolase. Gaschromatographic measurements of the main accumulating glycoprotein-derived metabolite, N-acetylglucosaminyl-asparagine (GlcNAc-Asn), in 12 defined cerebral areas showed that GlcNAc-Asn is rather evenly distributed in the brain. The mean concentrations ranged from 0.454 mg/g wet tissue (corpus callosum) to 0.0610 mg/g (pons). The GlcNAc-Asn concentrations tended to be higher in grey matter areas than in white matter areas. GlcNAc-Asn was identified in the isolated neuronal fraction, but not in the myelin fraction, by mass-fragmentographic techniques. Electron-microscopic reexamination of a brain biopsy specimen revealed, in addition to the abundant presence of storage lysosomes in the neuronal perikarya, numerous cytoplasmic inclusions in brain capillary endothelial cells and pericytes as well as in occasional macrophages. The results indicate that the glycoasparagine storage material is not limited to expected cortical areas in aspartylglycosaminuria, but is distributed in a rather constant fashion in all cerebral grey and white matter areas studied.

journal_name

J Neurol Sci

journal_title

Journal of the neurological sciences

authors

Maury CP,Haltia M,Palo J

doi

10.1016/0022-510x(81)90174-x

subject

Has Abstract

pub_date

1981-05-01 00:00:00

pages

291-8

issue

2

eissn

0022-510X

issn

1878-5883

pii

0022-510X(81)90174-X

journal_volume

50

pub_type

杂志文章

相关文献

JOURNAL OF THE NEUROLOGICAL SCIENCES文献大全
  • Intravenous thrombolysis followed by intra-arterial thrombolysis and mechanical thrombectomy for the treatment of pediatric ischemic stroke.

    abstract：:Experience with systemic or selective local administration of thrombolytic agents in pediatric ischemic stroke is limited to sporadic case reports, since patients of age less than 18 years were systematically excluded from randomised controlled trials. We report a case of childhood IS attributable to the terminal inte...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2008.07.018

    authors： Tsivgoulis G,Horton JA,Ness JM,Patterson D,Brethour M,Abanses JC,Alexandrov AV

    更新日期：2008-12-15 00:00:00

  • A longitudinal study of immunological parameters in multiple sclerosis. Cytotoxic antibodies.

    abstract：:Complement-dependent gliotoxic antibody activity was determined in 22 patients with multiple sclerosis (MS) and 19 normal control persons. Peripheral blood serum was collected from MS patients at about 4-week intervals for one year, and the results of cytotoxicity tests correlated with the course of disease. For 10 MS...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/0022-510x(79)90096-0

    authors： Mar P,Gradl T,Dörner C

    更新日期：1979-05-01 00:00:00

  • Utility of T2-weighted high-resolution vessel wall imaging for the diagnosis of isolated posterior inferior cerebellar artery dissection at acute and early subacute stages.

    abstract：:Recent advances in magnetic resonance high-resolution vessel wall imaging (HRVWI), which can detect intramural hematomas (IMH), improve the noninvasive diagnostic accuracy of isolated posterior inferior cerebellar artery dissection (iPICA-D). However, despite the risk of overlooking minute IMH, the utility of T2-weigh...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2020.116693

    authors： Kano Y,Inui S,Oguri T,Kato H,Yuasa H,Morimoto S,Sakurai K

    更新日期：2020-04-15 00:00:00

  • Electron-microscopic X-ray microanalysis of normal and diseased human muscle.

    abstract：:Electron-microscopic X-ray microanalysis has been used to compare elemental concentrations in specific organelles in normal and diseased human muscle. An elevated calcium to phosphorus ratio has been found in both myonuclei and interstitial cell nuclei in diseased muscle compared with controls. Preliminary observation...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/0022-510x(77)90129-0

    authors： Maunder CA,Yarom R,Dubowitz V

    更新日期：1977-09-01 00:00:00

  • Neurotransmitter amino acids in cerebrospinal fluid of patients with Parkinson's disease.

    abstract：:We measured the CSF and plasma levels of glutamate, glutamine, aspartate (only in plasma), asparagine, glutamine, glycine and GABA in 31 patients with Parkinson's disease and in 45 matched controls. We used an ion-exchange chromatography method. When compared to controls, PD patients had similar CSF levels of glutamat...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/0022-510x(96)00115-3

    authors： Jiménez-Jiménez FJ,Molina JA,Vargas C,Gómez P,Navarro JA,Benito-León J,Ortí-Pareja M,Gasalla T,Cisneros E,Arenas J

    更新日期：1996-09-15 00:00:00

  • Rapid ultrasonographic diagnosis of radial entrapment neuropathy at the spiral groove.

    abstract：BACKGROUND:Entrapment neuropathy of the radial nerve at the spiral groove region is relatively common. However, its localization may be technically challenging. OBJECTIVE:To evaluate the use of ultrasound (US), in relation to electrophysiological testing, for this purpose. METHODS:We studied 32 normal controls to obt...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2008.03.014

    authors： Lo YL,Fook-Chong S,Leoh TH,Dan YF,Tan YE,Lee MP,Gan HY,Chan LL

    更新日期：2008-08-15 00:00:00

  • Meiotic instability of the CAG repeats in the SCA6/CACNA1A gene in two Japanese SCA6 families.

    abstract：:Intergenerational stability of the CAG repeat number has been considered to be a specific molecular feature of SCA6 compared with other CAG repeat diseases. Nevertheless, we showed meiotic instability of the CAG repeats in the SCA6/CACNL1A gene in two Japanese SCA6 families, including de novo expansion. In one family,...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/s0022-510x(01)00466-x

    authors： Shimazaki H,Takiyama Y,Sakoe K,Amaike M,Nagaki H,Namekawa M,Sasaki H,Nakano I,Nishizawa M

    更新日期：2001-04-01 00:00:00

  • Clinical and neuroimaging features of the m.10197G>A mtDNA mutation: New case reports and expansion of the phenotype variability.

    abstract：:Complex I (CI) is the largest component of the mitochondrial respiratory chain (MRC) and it is made up of 7 mitochondrial DNA (mtDNA)-encoded and at least 38 nuclear DNA-encoded subunits. Isolated CI deficiency is the most common single enzyme deficiency in the heterogeneous group of MRC disorders and it is a relative...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2019.02.010

    authors： Tolomeo D,Rubegni A,Severino M,Pochiero F,Bruno C,Cassandrini D,Madeo A,Doccini S,Pedemonte M,Rossi A,D'Amore F,Donati MA,Di Rocco M,Santorelli FM,Nesti C

    更新日期：2019-04-15 00:00:00

  • Location-specific characteristics of perivascular spaces as the brain's interstitial fluid drainage system.

    abstract：PURPOSE:Brain interstitial fluid plays an important role in the excretion of metabolic waste products into the cerebrospinal fluid through perivascular spaces (PVS). To investigate the normal function of PVS in healthy elderly individuals, we assessed the relationship between PVS and white matter hyperintensity (WMH) o...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2019.01.022

    authors： Yamada S,Ishikawa M,Yamamoto K,Yamaguchi M,Oshima M

    更新日期：2019-03-15 00:00:00

  • Differentiating progressive supranuclear palsy from Parkinson's disease by MRI-based dynamic cerebrospinal fluid flow.

    abstract：OBJECTIVE:The purpose of this study was to clarify the difference between PSP and PD from the viewpoint of dynamic cerebrospinal fluid (CSF) flow focusing on the midbrain aqueduct. METHODS:Thirty-three PD patients (mean age 69.2±7.9) and 35 PSP patients (mean age 70.5±6.6) were included in this study. CSF flow was cal...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2015.07.026

    authors： Fukui Y,Hishikawa N,Sato K,Yunoki T,Kono S,Matsuzono K,Nakano Y,Ohta Y,Yamashita T,Deguchi K,Abe K

    更新日期：2015-10-15 00:00:00

  • Neuroprotective effects of Nigella sativa extract on cell death in hippocampal neurons following experimental global cerebral ischemia-reperfusion injury in rats.

    abstract：BACKGROUND:Global cerebral ischemia followed by reperfusion, leads to extensive neuronal damage, particularly the neurons in the hippocampal CA region. Recent studies have demonstrated that pharmacological agents, such as Nigella sativa L. (Ranunculaceae) that is an annual herbaceous flowering plant, given at the time ...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2013.11.019

    authors： Hobbenaghi R,Javanbakht J,Sadeghzadeh Sh,Kheradmand D,Abdi FS,Jaberi MH,Mohammadiyan MR,Khadivar F,Mollaei Y

    更新日期：2014-02-15 00:00:00

  • Frontal-lobe syndrome and psychosis after damage to the brainstem dopaminergic nuclei.

    abstract：:A patient developed frontal-lobe syndrome and psychotic symptoms after infarction in the pontomesencephalic junction. Stereotaxic lesion localization on magnetic resonance imaging and statistical analyses of regional cerebral blood flow (rCBF) disclosed an involvement of the rostral brainstem dopaminergic nuclei and h...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2007.04.021

    authors： Nishio Y,Ishii K,Kazui H,Hosokai Y,Mori E

    更新日期：2007-09-15 00:00:00

  • Bilateral claw hand: an uncommon presentation of regional Guillain-Barré syndrome.

    abstract：:We present an uncommon case of a 38-year-old man presented with bilateral subacute weakness of intrinsic hand muscles, manifesting as bilateral claw-hand, without sensory deficits and absent tendon reflexes in upper arms. Nerve conduction studies showed findings consistent with demyelinating GBS. During the fourth day...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2013.07.009

    authors： Tsivgoulis G,Tsakaldimi S,Vadikolias K,Mantatzis M,Katsanos AH,Heliopoulos I,Piperidou C

    更新日期：2013-11-15 00:00:00

  • Practical issues in measuring autoantibodies to neuronal cell-surface antigens in autoimmune neurological disorders: 190 cases.

    abstract：OBJECTIVES:To address practical issues in measuring autoantibodies to neuronal cell-surface antigens (NSAs) in various autoimmune neurological disorders (ANDs). METHODS:We retrospectively reviewed the clinical information of 221 patients with clinically suspected ANDs who underwent antibody testing for NSAs between Ja...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2018.04.009

    authors： Kaneko J,Kanazawa N,Tominaga N,Kaneko A,Suga H,Usui R,Ishima D,Kitamura E,Akutsu T,Yoshida K,Nishiyama K,Iizuka T

    更新日期：2018-07-15 00:00:00

  • Cerebro- and cardiovascular reactivity and neuropsychological performance in hypertensive patients.

    abstract：BACKGROUND:Hypertensive (HT) patients are at higher risk of cognitive decline than normotensive individuals, because high blood pressure is a risk factor for mild cognitive deterioration. In this study cardio- and cerebrovascular reactivity along with cognitive performance was assessed on newly diagnosed HT patients. ...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2010.07.022

    authors： Kovács KR,Szekeres CC,Bajkó Z,Csapó K,Molnár S,Oláh L,Magyar MT,Bereczki D,Kardos L,Soltész P,Bojtor AB,Csiba L

    更新日期：2010-12-15 00:00:00

  • Blast-induced moderate neurotrauma (BINT) elicits early complement activation and tumor necrosis factor α (TNFα) release in a rat brain.

    abstract：:Blast-induced neurotrauma (BINT) is a major medical concern yet its etiology is largely undefined. Complement activation may play a role in the development of secondary injury following traumatic brain injury; however, its role in BINT is still undefined. The present study was designed to characterize the complement s...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2012.02.002

    authors： Dalle Lucca JJ,Chavko M,Dubick MA,Adeeb S,Falabella MJ,Slack JL,McCarron R,Li Y

    更新日期：2012-07-15 00:00:00

  • Has COVID-19 played an unexpected "stroke" on the chain of survival?

    abstract：BACKGROUND:The COVID-19 pandemics required several changes in stroke management and it may have influenced some clinical or functional characteristics. We aimed to evaluate the effects of the COVID-19 pandemics on stroke management during the first month of Italy lockdown. In addition, we described the emergency struct...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2020.116889

    authors： Naccarato M,Scali I,Olivo S,Ajčević M,Buoite Stella A,Furlanis G,Lugnan C,Caruso P,Peratoner A,Cominotto F,Manganotti P

    更新日期：2020-07-15 00:00:00

  • Imbalance of interneuron distribution between neocortex and basal ganglia: consideration of epileptogenesis of focal cortical dysplasia.

    abstract：AIM:The balance of excitation and inhibition of neurons and neuronal network is very important to perform complete neuronal function. Damage or loss of inhibitory γ-aminobutyric acid (GABA)-ergic interneuron is associated with impaired inhibitory control of cortical pyramidal neurons, leading to hyperexcitability and e...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2012.08.035

    authors： Sakakibara T,Sukigara S,Otsuki T,Takahashi A,Kaneko Y,Kaido T,Saito Y,Sato N,Nakagawa E,Sugai K,Sasaki M,Goto Y,Itoh M

    更新日期：2012-12-15 00:00:00

  • Cardiovascular event rates in patients with cerebrovascular disease and atherothrombosis at other vascular locations: results from 1-year outcomes in the Japanese REACH Registry.

    abstract：:The REduction of Atherothrombosis for Continued Health (REACH) Registry is a large, international, prospective cohort of patients with atherothrombosis or multiple (>or=3) risk factors (MRFs) for atherothrombosis. Japanese patients (n=5193) were enrolled into the REACH registry between August and December 2004. One-ye...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章,多中心研究

    doi：10.1016/j.jns.2009.09.011

    authors： Uchiyama S,Goto S,Matsumoto M,Nagai R,Origasa H,Yamazaki T,Shigematsu H,Shimada K,Yamada N,Bhatt DL,Steg PG,Ikeda Y,REduction of Atherothrombosis for Continued Health Registry Investigators.

    更新日期：2009-12-15 00:00:00

  • Impact of cladribine therapy on changes in circulating dendritic cell subsets, T cells and B cells in patients with multiple sclerosis.

    abstract：BACKGROUND:Cladribine causes sustained reduction in peripheral T and B cell populations while sparing other immune cells. We determined two populations of dendritic cells (DCs): namely CD1c(+)/CD19(-) (myeloid DCs) and CD303(+)/CD123(+) (plasmacytoid DCs), CD19(+) B lymphocytes, CD3(+) T lymphocytes and CD4(+) or CD8(+...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2013.06.003

    authors： Mitosek-Szewczyk K,Tabarkiewicz J,Wilczynska B,Lobejko K,Berbecki J,Nastaj M,Dworzanska E,Kolodziejczyk B,Stelmasiak Z,Rolinski J

    更新日期：2013-09-15 00:00:00

  • Motor neuron disease in the province of Turin, Italy, 1966-1980. Survival analysis in an unselected population.

    abstract：:We performed a population based survival analysis of all incident cases (220) of motor neuron disease (MND) in the province of Turin, Italy, during the period 1966-1980. 175 cases were diagnosed as amyotrophic lateral sclerosis (ALS), 43 as progressive muscular atrophy (PMA) and 2 as progressive bulbar palsy (PBP). Th...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/0022-510x(84)90004-2

    authors： Mortara P,Chiò A,Rosso MG,Leone M,Schiffer D

    更新日期：1984-11-01 00:00:00

  • Unusual features of the Guillain-Barré syndrome after rabies vaccine prepared in suckling mouse brain.

    abstract：:Five patients with acute polyneuritis following treatment with rabies vaccine prepared in suckling mouse brains are reported. These patients had severe and protracted disease. The extensive cranial nerve involvement, high mortality rate, and disabling longterm sequelae were in contrast to cases of the Guillain-Barré s...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/0022-510x(87)90099-2

    authors： Cabrera J,Griffin DE,Johnson RT

    更新日期：1987-11-01 00:00:00

  • Enrichment of high mannose-type glycans in nervous tissue glycoproteins in neuronal ceroid-lipofuscinosis.

    abstract：:In view of the hypothesis that a biochemical abnormality in the childhood forms of neuronal ceroid-lipofuscinosis may lie in the utilization of dolichols in glycoprotein synthesis, we analyzed the oligosaccharide structures of brain glycoproteins in infantile neuronal ceroid-lipofuscinosis (INCL). Lectin affinity chro...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/0022-510x(86)90030-4

    authors： Krusius T,Viitala J,Palo J,Maury CP

    更新日期：1986-01-01 00:00:00

  • Effects of naftopidil on inhibitory transmission in substantia gelatinosa neurons of the rat spinal dorsal horn in vitro.

    abstract：OBJECTIVE:Naftopidil is used clinically for the treatment of voiding disorders in benign prostatic hyperplasia. Previous in vivo experiments in which naftopidil was applied intrathecally abolished rhythmic bladder contraction, suggesting that naftopidil might inhibit a voiding reflex through interaction with spinal dor...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2017.07.030

    authors： Uta D,Xie DJ,Hattori T,Kasahara KI,Yoshimura M

    更新日期：2017-09-15 00:00:00

  • Phenotypical features of two patients diagnosed with PHARC syndrome and carriers of a new homozygous mutation in the ABHD12 gene.

    abstract：:PHARC (Polyneuropathy, Hearing loss, Ataxia, Retinitis pigmentosa and Cataracts) (MIM# 612674) is an autosomal recessive neurodegenerative disease caused by mutations in the ABHD12 gene. We evaluated two Spanish siblings affected with pes cavus, sensorimotor neuropathy, hearing loss, retinitis pigmentosa and juvenile ...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2018.02.021

    authors： Frasquet M,Lupo V,Chumillas MJ,Vázquez-Costa JF,Espinós C,Sevilla T

    更新日期：2018-04-15 00:00:00

  • Changes in risk factors and preventive treatments by stroke subtypes over 20 years: a population-based study.

    abstract：:In order to evaluate progress in preventive therapeutic strategies, and to identify potential targets for improvement, it is essential to assess changes in the risk factors and preventive treatment of stroke over time. All of the strokes occurring within the population of Dijon, France (150,000 inhabitants), were pros...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2009.08.062

    authors： Béjot Y,Osseby GV,Gremeaux V,Durier J,Rouaud O,Moreau T,Giroud M

    更新日期：2009-12-15 00:00:00

  • Hemorheologic profiles of plasma fibrinogen and blood viscosity from silent to acute and chronic cerebral infarctions.

    abstract：:Hemorheologic changes from silent to acute and chronic cerebral infarction have seldom been reported. We evaluated hemorheologic profiles of the whole blood viscosity, plasma viscosity and fibrinogen level in stroke at-risk patients with silent cerebral infarction, patients with acute or chronic cerebral lacunar infar...

    journal_title：Journal of the neurological sciences

    pub_type： 临床试验,杂志文章

    doi：10.1016/s0022-510x(96)05309-9

    authors： Tsuda Y,Satoh K,Kitadai M,Takahashi T

    更新日期：1997-03-20 00:00:00

  • Prevalence of MR imaging abnormalities in vitamin B12 deficiency patients presenting with clinical features of subacute combined degeneration of the spinal cord.

    abstract：BACKGROUND AND AIMS:Subacute combined degeneration (SACD) of the spinal cord, characterized by degeneration of lateral and posterior columns, is often found in vitamin B12 deficiency. Our aim was to look for sensitivity of imaging in depicting the spinal cord abnormality in vitamin B12 deficient patients and to find an...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/j.jns.2014.05.020

    authors： Jain KK,Malhotra HS,Garg RK,Gupta PK,Roy B,Gupta RK

    更新日期：2014-07-15 00:00:00

  • Experimental ischemic myopathy. Effects of aortic ligation and serotonin.

    abstract：:Support for the hypothesis that biogenic amines are involved in the production of muscle diseases comes from a report that rat muscle is damaged by combining distal aortic ligation with serotonin injection. Our studies explore the role of serotonin in the production of the myopathic changes in the aortic ligation-sero...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/0022-510x(79)90005-4

    authors： Kelts KA,Kaiser KK

    更新日期：1979-01-01 00:00:00

  • Axonal damage in multiple sclerosis plaques: a combined magnetic resonance imaging and 1H-magnetic resonance spectroscopy study.

    abstract：:The purpose of this study was to compare magnetic resonance imaging (MRI) features and proton MR spectroscopy (1H-MRS) patterns of multiple sclerosis (MS) plaques in order to define the metabolic substrate in different lesion subtypes. Combined MRI and single-voxel 1H-MRS investigation was performed in 54 MS patients ...

    journal_title：Journal of the neurological sciences

    pub_type： 杂志文章

    doi：10.1016/s0022-510x(00)00464-0

    authors： Simone IL,Tortorella C,Federico F,Liguori M,Lucivero V,Giannini P,Carrara D,Bellacosa A,Livrea P

    更新日期：2001-01-01 00:00:00