Deferiprone targets aconitase: implication for Friedreich's ataxia treatment.

Abstract:

BACKGROUND:Friedreich ataxia is a neurological disease originating from an iron-sulfur cluster enzyme deficiency due to impaired iron handling in the mitochondrion, aconitase being particularly affected. As a mean to counteract disease progression, it has been suggested to chelate free mitochondrial iron. Recent years have witnessed a renewed interest in this strategy because of availability of deferiprone, a chelator preferentially targeting mitochondrial iron. METHOD:Control and Friedreich's ataxia patient cultured skin fibroblasts, frataxin-depleted neuroblastoma-derived cells (SK-N-AS) were studied for their response to iron chelation, with a particular attention paid to iron-sensitive aconitase activity. RESULTS:We found that a direct consequence of chelating mitochondrial free iron in various cell systems is a concentration and time dependent loss of aconitase activity. Impairing aconitase activity was shown to precede decreased cell proliferation. CONCLUSION:We conclude that, if chelating excessive mitochondrial iron may be beneficial at some stage of the disease, great attention should be paid to not fully deplete mitochondrial iron store in order to avoid undesirable consequences.

journal_name

BMC Neurol

journal_title

BMC neurology

authors

Goncalves S,Paupe V,Dassa EP,Rustin P

doi

10.1186/1471-2377-8-20

subject

Has Abstract

pub_date

2008-06-16 00:00:00

pages

20

issn

1471-2377

pii

1471-2377-8-20

journal_volume

8

pub_type

杂志文章
  • Delayed extensive brain edema caused by the growth of a giant basilar apex aneurysm treated with basilar artery obliteration: a case report.

    abstract:BACKGROUND:Partially thrombosed giant aneurysms at the basilar apex (BA) artery are challenging lesions with a poor prognosis if left untreated. Here we describe a rare case of extensive brain edema after growth of a surgically treated and thrombosed giant basilar apex aneurysm. CASE PRESENTATION:We performed a proxim...

    journal_title:BMC neurology

    pub_type: 杂志文章

    doi:10.1186/s12883-020-01819-9

    authors: García-Pérez D,Panero I,Eiriz C,Moreno LM,Munarriz PM,Paredes I,Lagares A,Alén JF

    更新日期:2020-06-06 00:00:00

  • Selection bias in clinical stroke trials depending on ability to consent.

    abstract:BACKGROUND:Clinical trials are the hallmark of evidence-based medicine, but recruitment is often challenging, especially in stroke trials investigating patients not being able to give informed consent. In some nations, ethics committees will not approve of inclusion in a clinical study via consent of a legal representa...

    journal_title:BMC neurology

    pub_type: 杂志文章,多中心研究,随机对照试验

    doi:10.1186/s12883-017-0989-9

    authors: Hotter B,Ulm L,Hoffmann S,Katan M,Montaner J,Bustamante A,Meisel A

    更新日期:2017-12-04 00:00:00

  • Leg orientation as a clinical sign for pusher syndrome.

    abstract:BACKGROUND:Effective control of (upright) body posture requires a proper representation of body orientation. Stroke patients with pusher syndrome were shown to suffer from severely disturbed perception of own body orientation. They experience their body as oriented 'upright' when actually tilted by nearly 20 degrees to...

    journal_title:BMC neurology

    pub_type: 杂志文章

    doi:10.1186/1471-2377-6-30

    authors: Johannsen L,Broetz D,Karnath HO

    更新日期:2006-08-23 00:00:00

  • Anti-N-methyl-D-aspartate receptor(NMDAR) antibody encephalitis presents in atypical types and coexists with neuromyelitis optica spectrum disorder or neurosyphilis.

    abstract:BACKGROUND:Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a clinically heterogeneous disorder characterized by epileptic seizures, psychosis, dyskinesia, consciousness impairments, and autonomic instability. Symptoms are always various. Sometimes it presents in milder or incomplete forms. We report 4 cases ...

    journal_title:BMC neurology

    pub_type: 杂志文章

    doi:10.1186/s12883-016-0787-9

    authors: Qin K,Wu W,Huang Y,Xu D,Zhang L,Zheng B,Jiang M,Kou C,Gao J,Li W,Zhang J,Wang S,Luan Y,Yan C,Xu D,Zheng X

    更新日期:2017-01-05 00:00:00

  • Subarachnoid hemorrhage secondary to a ruptured middle cerebral aneurysm in a patient with osteogenesis imperfecta: a case report.

    abstract:BACKGROUND:Osteogenesis imperfecta (OI) is a heterogeneous group of inherited disorders that occur owing to the abnormalities in type 1 collagen, and is characterized by increased bone fragility and other extraskeletal manifestations. We report the case of a patient who was diagnosed with OI following subarachnoid hemo...

    journal_title:BMC neurology

    pub_type: 杂志文章

    doi:10.1186/1471-2377-14-150

    authors: Hirohata T,Miyawaki S,Mizutani A,Iwakami T,Yamada S,Nishido H,Suzuki Y,Miyamoto S,Hoya K,Murakami M,Matsuno A

    更新日期:2014-07-23 00:00:00

  • Comparison of repair of peripheral nerve transection in predegenerated muscle with and without a vein graft.

    abstract:BACKGROUND:Despite substantial research into the topic and valiant surgical efforts, reconstruction of peripheral nerve injury remains a challenging surgery. This study was conducted to evaluate the effectiveness of axonal regeneration of a transected sciatic nerve through a vein conduit containing degenerated skeletal...

    journal_title:BMC neurology

    pub_type: 杂志文章

    doi:10.1186/s12883-016-0768-z

    authors: Mohammadi J,Delaviz H,Mohammadi B,Delaviz H,Rad P

    更新日期:2016-11-22 00:00:00

  • Increased incidence of Susac syndrome: a case series study.

    abstract:BACKGROUND:Susac syndrome (SuS) is a rare condition characterized by a clinical triad of sensorineural hearing loss, branch artery occlusion and encephalopathy. This study reports an increased incidence of SuS in Israel. We describe the clinical characteristics of these patients, diagnostic procedures and the use and s...

    journal_title:BMC neurology

    pub_type: 杂志文章

    doi:10.1186/s12883-020-01892-0

    authors: Wilf-Yarkoni A,Elkayam O,Aizenstein O,Oron Y,Furer V,Zur D,Goldstein M,Barequet D,Hallevi H,Karni A,Habot-Wilner Z,Regev K

    更新日期:2020-09-02 00:00:00

  • Cost effectiveness of recombinant factor VIIa for treatment of intracerebral hemorrhage.

    abstract:BACKGROUND:Phase I/II placebo-controlled clinical trials of recombinant Factor VIIa (rFVIIa) suggested that administration of rFVIIa within 4 hours after onset of intracerebral hemorrhage (ICH) is safe, limits ICH growth, and improves outcomes. We sought to determine the cost-effectiveness of rFVIIa for acute ICH treat...

    journal_title:BMC neurology

    pub_type: 杂志文章

    doi:10.1186/1471-2377-8-17

    authors: Kissela BM,Eckman MH

    更新日期:2008-05-19 00:00:00

  • Neuropsychological outcome after carbon monoxide exposure following a storm: a case-control study.

    abstract:BACKGROUND:The cognitive consequences of carbon monoxide (CO) poisoning are well described. However, most studies have been carried out without an ad-hoc group of control subjects. The main aim of this study was to evaluate cognitive and psychiatric outcome after CO exposure during the storm Klaus in the South West of ...

    journal_title:BMC neurology

    pub_type: 杂志文章

    doi:10.1186/1471-2377-14-153

    authors: Pages B,Planton M,Buys S,Lemesle B,Birmes P,Barbeau EJ,Maziero S,Cordier L,Cabot C,Puel M,Genestal M,Chollet F,Pariente J

    更新日期:2014-07-21 00:00:00

  • Leukoencephalopathy resolution after atypical mycobacterial treatment: a case report.

    abstract:BACKGROUND:Association of leukoencephalopathy and atypical mycobacteriosis has been rarely reported. We present a case that is relevant for its unusual presentation and because it may shed further light on the pathogenic mechanisms underlying reversible encephalopathies. CASE REPORT:We report the case of a Hispanic 64...

    journal_title:BMC neurology

    pub_type: 杂志文章

    doi:10.1186/s12883-015-0415-0

    authors: Oliveira MC,Sato DK,Soares-Neto HR,Lucato LT,Callegaro D,Nitrini R,Medeiros RS,Misu T,Fujihara K,Castro LH

    更新日期:2015-09-02 00:00:00

  • Loss of circadian rhythm of blood pressure following acute stroke.

    abstract:BACKGROUND:Epidemiology of acute stroke in developing countries differs from that in the developed world, for example, the age at stroke, risk factors, subtypes of stroke and prognosis. Hypertension remains a dominant risk factor and prognostic indicator in patients with stroke in all communities. The risk of stroke is...

    journal_title:BMC neurology

    pub_type: 杂志文章

    doi:10.1186/1471-2377-4-1

    authors: Jain S,Namboodri KK,Kumari S,Prabhakar S

    更新日期:2004-01-06 00:00:00

  • Free radical scavenger, edaravone, reduces the lesion size of lacunar infarction in human brain ischemic stroke.

    abstract:BACKGROUND:Although free radicals have been reported to play a role in the expansion of ischemic brain lesions, the effect of free radical scavengers is still under debate. In this study, the temporal profile of ischemic stroke lesion sizes was assessed for more than one year to evaluate the effect of edaravone which m...

    journal_title:BMC neurology

    pub_type: 临床试验,杂志文章

    doi:10.1186/1471-2377-11-39

    authors: Nakase T,Yoshioka S,Suzuki A

    更新日期:2011-03-30 00:00:00

  • Chronic cerebrospinal venous insufficiency in multiple sclerosis: a highly prevalent age-dependent phenomenon.

    abstract:BACKGROUND:This study aimed to investigate the prevalence and clinical relevance of chronic cerebrospinal venous insufficiency (CCSVI) in multiple sclerosis (MS) patients and healthy controls using extra- and intracranial colour Doppler sonography. METHODS:We examined 146 MS patients, presenting with a clinically isol...

    journal_title:BMC neurology

    pub_type: 杂志文章

    doi:10.1186/1471-2377-13-20

    authors: Lanzillo R,Mancini M,Liuzzi R,Di Donato O,Salvatore E,Maglio V,Vacca G,Amato L,D'Anna G,Brunetti A,Brescia Morra V

    更新日期:2013-02-13 00:00:00

  • Basal ganglia hemorrhage in a case report following spinal surgery.

    abstract:BACKGROUND:Intracranial hemorrhage is a rare but potentially severe complication of spinal surgery. Most reported post-operative ICH cases consist of cerebellar hemorrhage. There are fewer reported cases of supratentorial ICH following spinal surgery. CASE PRESENTATION:A 56-year-old woman underwent spinal surgery comp...

    journal_title:BMC neurology

    pub_type: 杂志文章

    doi:10.1186/s12883-018-1218-x

    authors: Berry B,Ghannam M,Bell C,Ghazaleh S,Boss S,Streib C,Ezzeddine M

    更新日期:2018-12-14 00:00:00

  • Long-term effects of STN DBS on mood: psychosocial profiles remain stable in a 3-year follow-up.

    abstract:BACKGROUND:Deep brain stimulation of the subthalamic nucleus significantly improves motor function in patients with severe Parkinson's disease. However, the effects on nonmotor aspects remain uncertain. The present study investigated the effects of subthalamic nucleus deep brain stimulation on mood and psychosocial fun...

    journal_title:BMC neurology

    pub_type: 临床试验,杂志文章

    doi:10.1186/1471-2377-8-43

    authors: Kaiser I,Kryspin-Exner I,Brücke T,Volc D,Alesch F

    更新日期:2008-11-11 00:00:00

  • Taste disorder in facial onset sensory and motor neuronopathy: a case report.

    abstract:BACKGROUND:Taste disorder is a common symptom in the general population. Several studies have shown that patients with neurological disorders, such as amyotrophic lateral sclerosis and Parkinson's disease, develop taste disturbance. Facial onset sensory and motor neuronopathy (FOSMN) is a rare disease characterized by ...

    journal_title:BMC neurology

    pub_type: 杂志文章

    doi:10.1186/s12883-020-01639-x

    authors: Ohashi N,Nonami J,Kodaira M,Yoshida K,Sekijima Y

    更新日期:2020-02-29 00:00:00

  • The effects of a home-based arm ergometry exercise programme on physical fitness, fatigue and activity in polio survivors: protocol for a randomised controlled trial.

    abstract:BACKGROUND:Many Polio survivors have reduced mobility, pain and fatigue, which make access to conventional forms of aerobic exercise difficult. Inactivity leads to increased risk of health problems, many of which are prevalent among Polio survivors. Aerobic exercise programmes in Polio survivors should utilise stable m...

    journal_title:BMC neurology

    pub_type: 杂志文章,随机对照试验

    doi:10.1186/1471-2377-12-157

    authors: Murray D,Meldrum D,Moloney R,Campion A,Horgan F,Hardiman O

    更新日期:2012-12-13 00:00:00

  • Management of oral anticoagulation after cardioembolic stroke and stroke survival data from a population based stroke registry (LuSSt).

    abstract:BACKGROUND:Cardioembolic stroke (CES) due to atrial fibrillation (AF) is associated with high stroke mortality. Oral anticoagulation (OAC) reduces stroke mortality, however, the impact of OAC-administration during hospital stay post ischemic stroke on mortality is unclear. We determined whether the timing of OAC initia...

    journal_title:BMC neurology

    pub_type: 杂志文章

    doi:10.1186/s12883-014-0199-7

    authors: Palm F,Kraus M,Safer A,Wolf J,Becher H,Grau AJ

    更新日期:2014-10-08 00:00:00

  • Anti-MOG antibody-positive ADEM following infectious mononucleosis due to a primary EBV infection: a case report.

    abstract:BACKGROUND:Anti-Myelin oligodendrocyte glycoprotein (MOG) antibodies are detected in various demyelinating diseases, such as pediatric acute disseminated encephalomyelitis (ADEM), recurrent optic neuritis, and aquaporin-4 antibody-seronegative neuromyelitis optica spectrum disorder. We present a patient who developed a...

    journal_title:BMC neurology

    pub_type: 杂志文章

    doi:10.1186/s12883-017-0858-6

    authors: Nakamura Y,Nakajima H,Tani H,Hosokawa T,Ishida S,Kimura F,Kaneko K,Takahashi T,Nakashima I

    更新日期:2017-04-19 00:00:00

  • Genetic analysis of spinal dysraphism with a hamartomatous growth (appendix) of the spinal cord: a case series.

    abstract:BACKGROUND:Spinal dysraphism with a hamartomatous growth (appendix) of the spinal cord is better known as herniated spinal cord. There are many arguments in favour of considering it a developmental defect. From this point of view, it is a type of neural tube disorder. Neural tube disorders can be caused by multiple fac...

    journal_title:BMC neurology

    pub_type: 杂志文章

    doi:10.1186/s12883-020-01710-7

    authors: Bartels RHMA,Grotenhuis JA,Stegmann APA,Brunner H

    更新日期:2020-04-06 00:00:00

  • Thermographic follow-up of postherpetic neuralgia (PHN) subsequent to Ramsay Hunt syndrome with multicranial nerve (V, VII, VIII and IX) involvement: a case report.

    abstract:BACKGROUND:Ramsay Hunt syndrome (RHS) is caused by a reactivation of varicella-zoster virus (VZV) infection, and it is characterized by the symptoms of facial paralysis, otalgia, auricular rash, and/or an oral lesion. Elderly patients or immunocompromised patients, deep pain at the initial visit and no prompt treatment...

    journal_title:BMC neurology

    pub_type: 杂志文章

    doi:10.1186/s12883-021-02071-5

    authors: Liao YM,Lu HF,Xie P,Zhao Y,Han Q,Zhang QX,Zuo XH,Si YN,Bao HG

    更新日期:2021-01-28 00:00:00

  • Evaluation of rotigotine transdermal patch for the treatment of apathy and motor symptoms in Parkinson's disease.

    abstract:BACKGROUND:This multicenter, double-blind, placebo-controlled study assessed the efficacy of rotigotine transdermal patch on apathy and motor symptoms in patients with Parkinson's disease (PD). METHODS:Patients with PD-associated apathy (Unified Parkinson's Disease Rating Scale [UPDRS] I item 4 [motivation] ≥2 and pat...

    journal_title:BMC neurology

    pub_type: 杂志文章,多中心研究,随机对照试验

    doi:10.1186/s12883-016-0610-7

    authors: Hauser RA,Slawek J,Barone P,Dohin E,Surmann E,Asgharnejad M,Bauer L

    更新日期:2016-06-07 00:00:00

  • Identification of peripheral inflammatory markers between normal control and Alzheimer's disease.

    abstract:BACKGROUND:Multiple pathogenic factors may contribute to the pathophysiology of Alzheimer's disease (AD). Peripheral blood markers have been used to assess biochemical changes associated with AD and mild cognitive impairment (MCI) and involved in their pathophysiology. METHODS:Plasma samples and clinical data were obt...

    journal_title:BMC neurology

    pub_type: 杂志文章

    doi:10.1186/1471-2377-11-51

    authors: Kim SM,Song J,Kim S,Han C,Park MH,Koh Y,Jo SA,Kim YY

    更新日期:2011-05-12 00:00:00

  • Decision-making under explicit risk is impaired in multiple sclerosis: relationships with ventricular width and disease disability.

    abstract:BACKGROUND:Decision-making is an essential function of everyday life. Decision-making under explicit risk requires developing advantageous decision strategies based on fixed outcomes (e.g., probabilities of winning or losing a bet). Decision-making and its neural substrates have been rarely studied in MS. We expected p...

    journal_title:BMC neurology

    pub_type: 杂志文章

    doi:10.1186/s12883-015-0318-0

    authors: Radomski AD,Power C,Purdon SE,Emery DJ,Blevins G,Warren KG,Fujiwara E

    更新日期:2015-04-23 00:00:00

  • Tapping-lips aggravated interictal bilateral discharges in EEG in the patients with Rett syndrome: a case report.

    abstract:BACKGROUND:Rett syndrome (RTT) is a severe neurodevelopmental disorder mainly affecting females. One of the main clinical manifestations is hand stereotypies, which is presumed to based on dysfunction rather than on structural impairment. Reflex interictal discharge precipitated by tapping-lips in electroencephalogram ...

    journal_title:BMC neurology

    pub_type: 杂志文章

    doi:10.1186/s12883-019-1296-4

    authors: Luo X,Li C,Wang X,Yu X,Jiang J

    更新日期:2019-05-01 00:00:00

  • Predictors and long-term outcome of seizures in human immuno-deficiency virus (HIV)-negative cryptococcal meningitis.

    abstract:BACKGROUND:Seizures are one of the most important neurologic complications of human immuno-deficiency virus (HIV)-negative cryptococcal meningitis. A better understanding of the risk associated factors can help predict those who will require treatment. METHODS:This 22-year retrospective study enrolled 180 patients. Pr...

    journal_title:BMC neurology

    pub_type: 杂志文章

    doi:10.1186/s12883-014-0208-x

    authors: Hung CW,Chang WN,Kung CT,Tsai NW,Wang HC,Lin WC,Huang CR,Huang CC,Tsai WC,Chang HW,Su YJ,Lin YJ,Cheng BC,Chang YT,Su CM,Lu CH

    更新日期:2014-10-13 00:00:00

  • Pretreatment with statins improves early outcome in patients with first-ever ischaemic stroke: a pleiotropic effect of statins or a beneficial effect of hypercholesterolemia?

    abstract:BACKGROUND:Data from different studies suggest a favourable association between pretreatment with statins or hypercholesterolemia and outcome after ischaemic stroke. We examined whether there were differences in in-hospital mortality according to the presence or absence of statin therapy in a large population of first-...

    journal_title:BMC neurology

    pub_type: 杂志文章

    doi:10.1186/1471-2377-10-47

    authors: Arboix A,García-Eroles L,Oliveres M,Targa C,Balcells M,Massons J

    更新日期:2010-06-18 00:00:00

  • Efficacy and acceptability of selective serotonin reuptake inhibitors for the treatment of depression in Parkinson's disease: a systematic review and meta-analysis of randomized controlled trials.

    abstract:BACKGROUND:Selective serotonin reuptake inhibitors (SSRIs) are the most commonly prescribed antidepressants for the treatment of depression in patients with Parkinson's Disease (PD) but data on their efficacy are controversial. METHODS:We conducted a systematic review and meta-analysis of randomized controlled trials ...

    journal_title:BMC neurology

    pub_type: 杂志文章,meta分析,评审

    doi:10.1186/1471-2377-10-49

    authors: Skapinakis P,Bakola E,Salanti G,Lewis G,Kyritsis AP,Mavreas V

    更新日期:2010-06-21 00:00:00

  • Improving the clinical assessment of consciousness with advances in electrophysiological and neuroimaging techniques.

    abstract::In clinical neurology, a comprehensive understanding of consciousness has been regarded as an abstract concept--best left to philosophers. However, times are changing and the need to clinically assess consciousness is increasingly becoming a real-world, practical challenge. Current methods for evaluating altered level...

    journal_title:BMC neurology

    pub_type: 杂志文章,评审

    doi:10.1186/1471-2377-10-11

    authors: Gawryluk JR,D'Arcy RC,Connolly JF,Weaver DF

    更新日期:2010-01-29 00:00:00

  • Head and neck CT angiography to assess the internal carotid artery stealing pathway.

    abstract:BACKGROUND:Common carotid artery occlusive disease (CCAOD) could form internal carotid artery steal pathways. Based on the diagnostic results of digital subtraction angiography (DSA), head and neck computed tomography angiography (CTA) was used to find the internal carotid artery stealing pathway after CCAOD. METHODS:...

    journal_title:BMC neurology

    pub_type: 杂志文章

    doi:10.1186/s12883-020-01915-w

    authors: Wang D,Li Z,Zheng X,Cong H,Zhang T,Wang Z,Wang Y,He J

    更新日期:2020-09-03 00:00:00