Review: Prion-like mechanisms of transactive response DNA binding protein of 43 kDa (TDP-43) in amyotrophic lateral sclerosis (ALS).

Abstract:

:Amyotrophic lateral sclerosis (ALS) is a fatal devastating neurodegenerative disorder which predominantly affects the motor neurons in the brain and spinal cord. The death of the motor neurons in ALS causes subsequent muscle atrophy, paralysis and eventual death. Clinical and biological evidence now demonstrates that ALS has many similarities to prion disease in terms of disease onset, phenotype variability and progressive spread. The pathognomonic ubiquitinated inclusions deposited in the neurons and glial cells in brains and spinal cords of patients with ALS and fronto-temporal lobar degeneration with ubiquitinated inclusions contain aggregated transactive response DNA binding protein of 43 kDa (TDP-43), and evidence now suggests that TDP-43 has cellular prion-like properties. The cellular mechanisms of prion protein misfolding and aggregation are thought to be responsible for the characteristics of prion disease. Therefore, there is a strong mechanistic basis for a prion-like behaviour of the TDP-43 protein being responsible for some characteristics of ALS. In this review, we compare the prion-like mechanisms of TDP-43 to the clinical and biological nature of ALS in order to investigate how this protein could be responsible for some of the characteristic properties of the disease.

authors

Smethurst P,Sidle KC,Hardy J

doi

10.1111/nan.12206

subject

Has Abstract

pub_date

2015-08-01 00:00:00

pages

578-97

issue

5

eissn

0305-1846

issn

1365-2990

journal_volume

41

pub_type

杂志文章,评审
  • Sequential studies of a childhood myopathy: a clinical, histochemical and morphometric investigation.

    abstract::An unusual inherited progressive distal myopathy of early childhood onset is described in two sisters from a consanguineous Asian family. Motor milestones were normal but gait deteriorated slowly thereafter with development of generalized hypotonia and muscle weakness particularly in the wrist extensors and hand muscl...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2990.1989.tb01145.x

    authors: Mahon M,Kristmundsdottir F,Cumming WJ,Noronha MJ

    更新日期:1989-01-01 00:00:00

  • Expression of Bcl-2 and Bax in oligodendrogliomas and their relationship to apoptosis.

    abstract::Apoptotic bodies are frequently found in oligodendrogliomas, particularly in the anaplastic subtype. A range of proteins, such as those of the Bcl family, are implicated in the control of apoptosis. The ratio of antagonists of apoptosis, such as Bcl-2, to agonists, such as Bax, is thought to determine the outcome for ...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2990.1999.00199.x

    authors: Delgado MB,Anderson JR,Whittle IR,Wharton SB

    更新日期:1999-10-01 00:00:00

  • Selective damage to the cerebellar vermis in chronic alcoholism: a contribution from neurotoxicology to an old problem of selective vulnerability.

    abstract::The curiously consistent localization of cerebellar cortical damage in chronic alcoholism is re-evaluated in the light of selective damage, with a similar topography in the cerebellar vermal region, in superficial siderosis in man and in experimental animals exposed to certain toxic substances. Attention is drawn to t...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 杂志文章,评审

    doi:

    authors: Cavanagh JB,Holton JL,Nolan CC

    更新日期:1997-10-01 00:00:00

  • Membranous expression of glucose transporter-1 protein (GLUT-1) in embryonal neoplasms of the central nervous system.

    abstract::The human erythrocyte GLUT-1 is a transmembrane protein which facilitates transport of glucose in the cell in an energy-independent fashion. Neuroectodermal stem cells show strong membrane immunoreactivitry with this marker at early developmental stages in rodents. Membranous expression by undifferentiated neuroectode...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 杂志文章

    doi:10.1046/j.1365-2990.2000.00225.x

    authors: Loda M,Xu X,Pession A,Vortmeyer A,Giangaspero F

    更新日期:2000-02-01 00:00:00

  • Loss of axonal contact causes down-regulation of the PLP gene in oligodendrocytes: evidence from partial lesions of the optic nerve.

    abstract::We have examined the influence that axons may have on the expression of proteolipid protein (PLP), the major myelin protein of the CNS. Partial transections were made of the optic nerve of adult rats to produce approximately a 50% loss of axons. Twenty-eight days after lesioning, sections of the distal nerves were imm...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2990.1991.tb00725.x

    authors: McPhilemy K,Griffiths IR,Mitchell LS,Kennedy PG

    更新日期:1991-08-01 00:00:00

  • Altered arginine metabolism in the central nervous system (CNS) of the Cln3-/- mouse model of juvenile Batten disease.

    abstract:BACKGROUND:Juvenile neuronal ceroid lipofuscinoses (JNCL) or juvenile Batten disease is a recessively inherited childhood neurodegenerative disorder resulting from a mutation in CLN3, which encodes a putative lysosomal protein of unknown function. AIM:Recent evidence suggests that a disruption in CLN3 function results...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2990.2008.00984.x

    authors: Chan CH,Ramirez-Montealegre D,Pearce DA

    更新日期:2009-04-01 00:00:00

  • Loss of neurofilaments in the neuromuscular junction in a rat model of proximal axonopathy.

    abstract:AIMS:Rodents exposed to 3,3'-iminodipropionitrile (IDPN) develop an axonopathy similar to that observed in amyotrophic lateral sclerosis motor neurones, in which neurofilaments accumulate in swollen proximal axon segments. This study addressed the hypotheses that this proximal axonopathy is associated with loss of neur...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2990.2011.01178.x

    authors: Soler-Martín C,Vilardosa U,Saldaña-Ruíz S,Garcia N,Llorens J

    更新日期:2012-02-01 00:00:00

  • Absence of neuronal nitric oxide synthase (nNOS) as a pathological marker for the diagnosis of Becker muscular dystrophy with rod domain deletions.

    abstract::Immunohistochemistry using antibodies to dystrophin is the pathological basis for the diagnosis of Duchenne and Becker muscular dystrophy (DMD and BMD). While the sarcolemma of DMD muscle is negative, BMD muscle generally shows variable labelling because of the translation of a partially functional dystrophin that is ...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2990.2004.00561.x

    authors: Torelli S,Brown SC,Jimenez-Mallebrera C,Feng L,Muntoni F,Sewry CA

    更新日期:2004-10-01 00:00:00

  • Immunohistochemical expression of trk receptor proteins in focal cortical dysplasia with intractable epilepsy.

    abstract::Focal cortical dysplasia (FCD), which is often associated with intractable epilepsy, is a form of abnormal structure of the cerebral cortex caused by a disorder of normal neocortical development. In such cerebral lesions obtained from four patients (two male, two female; average age 32.3 years at operation), the immun...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 临床试验,杂志文章

    doi:10.1046/j.1365-2990.1999.00180.x

    authors: Nishio S,Morioka T,Hamada Y,Hisada K,Fukui M

    更新日期:1999-06-01 00:00:00

  • Reduction and the intracellular translocation of EphB2 in Tg2576 mice and the effects of β-amyloid.

    abstract:AIMS:EphB2 is a member of receptor tyrosine kinases (RTKs) family that is essential for the cell adhesion, neural crest migration, axon guidance and synaptogenesis in the nervous system. Recent studies show that preservation of EphB2 in a transgenic mouse model of Alzheimer's disease (AD) rescues the cognitive deficit,...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 杂志文章

    doi:10.1111/nan.12019

    authors: Qu M,Jiang J,Liu XP,Tian Q,Chen LM,Yin G,Liu D,Wang JZ,Zhu LQ

    更新日期:2013-10-01 00:00:00

  • Review: Mechanistic target of rapamycin (mTOR) pathway, focal cortical dysplasia and epilepsy.

    abstract::Over the last decade, there has been increasing evidence that hyperactivation of the mechanistic target of rapamycin (mTOR) pathway is a hallmark of malformations of cortical development such as focal cortical dysplasia (FCD) or hemimegalencephaly. The mTOR pathway governs protein and lipid synthesis, cell growth and ...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 杂志文章,评审

    doi:10.1111/nan.12463

    authors: Marsan E,Baulac S

    更新日期:2018-02-01 00:00:00

  • Cytoplasmic intermediate filaments in cultured glial cells.

    abstract::Monolayer cultures of rat glial cells derived from 12 to 14 day fetal spinal cords and rat C6 glioma cells were examined for immunofluorescent reactivity with human or rabbit autoantibodies to intermediate filaments and with rabbit antibody to GFAP. The embryonic glial cells and C6 glima cells reacted identically with...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2990.1980.tb00213.x

    authors: Sotelo J,Toh BH,Lolait SJ,Yildiz A,Osung O,Holborow EJ

    更新日期:1980-07-01 00:00:00

  • Telomeric alterations in the default mode network during the progression of Alzheimer's disease: Selective vulnerability of the precuneus.

    abstract:AIMS:Although telomere length (TL) and telomere maintenance proteins (shelterins) are markers of cellular senescence and peripheral blood biomarkers of Alzheimer's disease (AD), little information is available on telomeric alterations during the prodromal stage (MCI) of AD. We investigated TL in the default mode networ...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 杂志文章

    doi:10.1111/nan.12672

    authors: Mahady LJ,He B,Malek-Ahmadi M,Mufson EJ

    更新日期:2020-10-26 00:00:00

  • Amyloid beta peptide causes chronic glial cell activation and neuro-degeneration after intravitreal injection.

    abstract::We have previously demonstrated that amyloid beta (Abeta) peptide is acutely toxic to retinal neurones in vivo and that this toxicity is mediated by an indirect mechanism. We have now extended these studies to look at the chronic effect of intravitreal injection of Abeta peptides on retinal ganglion cells (RGC), the p...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2990.2005.00666.x

    authors: Walsh DT,Bresciani L,Saunders D,Manca MF,Jen A,Gentleman SM,Jen LS

    更新日期:2005-10-01 00:00:00

  • The HSPB8-BAG3 chaperone complex is upregulated in astrocytes in the human brain affected by protein aggregation diseases.

    abstract:AIMS:HSPB8 is a small heat shock protein that forms a complex with the co-chaperone BAG3. Overexpression of the HSPB8-BAG3 complex in cells stimulates autophagy and facilitates the clearance of mutated aggregation-prone proteins, whose accumulation is a hallmark of many neurodegenerative disorders. HSPB8-BAG3 could thu...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2990.2011.01198.x

    authors: Seidel K,Vinet J,Dunnen WF,Brunt ER,Meister M,Boncoraglio A,Zijlstra MP,Boddeke HW,Rüb U,Kampinga HH,Carra S

    更新日期:2012-02-01 00:00:00

  • Toll-like receptor-4-mediated autophagy contributes to microglial activation and inflammatory injury in mouse models of intracerebral haemorrhage.

    abstract:AIMS:Much evidence demonstrates that Toll-like receptor-4 (TLR4)-mediated microglial activation is an important contributor to the inflammatory injury in intracerebral haemorrhage (ICH). However, the exact mechanism of TLR4-mediated microglial activation induced by ICH is not clear. In addition, microglial autophagy is...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 杂志文章

    doi:10.1111/nan.12177

    authors: Yang Z,Liu B,Zhong L,Shen H,Lin C,Lin L,Zhang N,Yuan B

    更新日期:2015-06-01 00:00:00

  • Steroid hormone receptors in pituitary adenomas: a biochemical, immunohistochemical and morphometric study on cryostat sections.

    abstract::Oestrogen receptors and progesterone receptors were measured by an isoelectric focussing technique in cytosols from cryostat sections of eight human pituitary adenomas. Cryostat sections adjacent to the assayed sample were stained for anterior pituitary hormones using the peroxidase-antiperoxidase technique, and the c...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2990.1986.tb00158.x

    authors: Ironside JW,Dangerfield VJ,Timperley WR,Underwood JC

    更新日期:1986-11-01 00:00:00

  • Subependymal cells provide a faster response to ependymal injury than astrocytes in the hydrocephalic brain.

    abstract::Obstructive hydrocephalus was induced in 12-day-old rats by the infusion of kaolin into the cisterna magna. After 7 days, cortical and ependymal lesions were made with thin wire in non-hydrocephalic brains and in hydrocephalic brains. The wounds were allowed to heal for times ranging from 1 day to 10 days post-lesion,...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2990.1992.tb00800.x

    authors: Collins P,Goulding DA

    更新日期:1992-08-01 00:00:00

  • Secreted protein, acidic and rich in cysteine (SPARC) expression in astrocytic tumour cells negatively correlates with proliferation, while vascular SPARC expression is associated with patient survival.

    abstract:AIMS:Secreted protein, acidic and rich in cysteine (SPARC) is a regulator of cell-matrix interaction and has been associated with tumour stage and patient survival in various malignancies. As no large-scale study has yet been undertaken, we investigated human brain and astrocytomas for SPARC expression and associations...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2990.2010.01072.x

    authors: Capper D,Mittelbronn M,Goeppert B,Meyermann R,Schittenhelm J

    更新日期:2010-04-01 00:00:00

  • Lipid alterations in human frontal cortex in ALS-FTLD-TDP43 proteinopathy spectrum are partly related to peroxisome impairment.

    abstract:AIM:Peroxisomes play a key role in lipid metabolism, and peroxisome defects have been associated with neurodegenerative diseases such as X-adrenoleukodystrophy and Alzheimer's disease. This study aims to elucidate the contribution of peroxisomes in lipid alterations of area 8 of the frontal cortex in the spectrum of TD...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 杂志文章

    doi:10.1111/nan.12681

    authors: Andrés-Benito P,Gelpi E,Jové M,Mota-Martorell N,Obis È,Portero-Otin M,Povedano M,Pujol A,Pamplona R,Ferrer I

    更新日期:2020-12-17 00:00:00

  • Single dose of MDMA causes extensive decrement of serotoninergic fibre density without blockage of the fast axonal transport in Dark Agouti rat brain and spinal cord.

    abstract::Prolonged neurotoxicity of the recreational drug, MDMA (3,4-methylenedioxymethamphetamine) on serotoninergic axon terminals has been suggested. The effect of a single (15 mg/kg) dose of intraperitoneally administered MDMA on serotoninergic fibre density, defined by tryptophan hydroxylase (TpH) and serotonin transporte...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2990.2006.00790.x

    authors: Kovács GG,Andó RD,Adori C,Kirilly E,Benedek A,Palkovits M,Bagdy G

    更新日期:2007-04-01 00:00:00

  • Ultrastructural localization of adhalin, alpha-dystroglycan and merosin in normal and dystrophic muscle.

    abstract::Adhalin and alpha-dystroglycan are two components of a complex of proteins that, in conjunction with dystrophin, provide a link between the subsarcolemmal cytoskeleton and the basal lamina of the extracellular matrix of skeletal muscle. In the absence of dystrophin, in Duchenne muscular dystrophy (DMD) and the mdx mou...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 杂志文章

    doi:

    authors: Cullen MJ,Walsh J,Roberds SL,Campbell KP

    更新日期:1996-02-01 00:00:00

  • Childhood spinal muscular atrophy induces alterations in contractile and regulatory protein isoform expressions.

    abstract:AIMS:Although modifications of the survival motor neurone gene are responsible for most spinal muscular atrophy (SMA) cases, the molecular pathophysiology and the muscular target proteins involved are still unknown. The aim of this study was to compare the expression of contractile and regulatory protein isoforms in qu...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2990.2008.00950.x

    authors: Stevens L,Bastide B,Maurage CA,Dupont E,Montel V,Cieniewski-Bernard C,Cuisset JM,Vallée L,Mounier Y

    更新日期:2008-12-01 00:00:00

  • GDNF-treated acellular nerve graft promotes motoneuron axon regeneration after implantation into cervical root avulsed spinal cord.

    abstract:UNLABELLED:It is well known that glial cell line-derived neurotrophic factor (GDNF) is a potent neurotrophic factor for motoneurons. We have previously shown that it greatly enhanced motoneuron survival and axon regeneration after implantation of peripheral nerve graft following spinal root avulsion. AIMS:In the curre...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2990.2012.01253.x

    authors: Chu TH,Wang L,Guo A,Chan VW,Wong CW,Wu W

    更新日期:2012-12-01 00:00:00

  • Invited Review: Emerging functions of histone H3 mutations in paediatric diffuse high-grade gliomas.

    abstract::Paediatric diffuse high-grade gliomas (pHGG) are rare, but deadly tumours. The discovery of recurrent mutations in the tail of histone H3, changing lysine 27 to methionine, or glycine 34 to arginine or valine, has illuminated a critical role for epigenetic dysregulation in the aetiology of childhood gliomas and opened...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 杂志文章,评审

    doi:10.1111/nan.12591

    authors: Kasper LH,Baker SJ

    更新日期:2020-02-01 00:00:00

  • RAPGEF2 mediates oligomeric Aβ-induced synaptic loss and cognitive dysfunction in the 3xTg-AD mouse model of Alzheimer's disease.

    abstract:AIMS:Amyloid-β (Aβ) oligomers trigger synaptic degeneration that precedes plaque and tangle pathology. However, the signalling molecules that link Aβ oligomers to synaptic pathology remain unclear. Here, we addressed the potential role of RAPGEF2 as a novel signalling molecule in Aβ oligomer-induced synaptic and cognit...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 杂志文章

    doi:10.1111/nan.12686

    authors: Jang YN,Jang H,Kim GH,Noh JE,Chang KA,Lee KJ

    更新日期:2020-12-20 00:00:00

  • Ex vivo study of dentate gyrus neurogenesis in human pharmacoresistant temporal lobe epilepsy.

    abstract:AIMS:Neurogenesis in adult humans occurs in at least two areas of the brain, the subventricular zone of the telencephalon and the subgranular layer of the dentate gyrus in the hippocampal formation. We studied dentate gyrus subgranular layer neurogenesis in patients subjected to tailored antero-mesial temporal resectio...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2990.2010.01102.x

    authors: Paradisi M,Fernández M,Del Vecchio G,Lizzo G,Marucci G,Giulioni M,Pozzati E,Antonelli T,Lanzoni G,Bagnara GP,Giardino L,Calzà L

    更新日期:2010-10-01 00:00:00

  • Peculiar changes in Rosenthal fibres in an atypical astrocytoma.

    abstract::A 50-year-old female patient died of an untreatable glioma apoplecticum. At autopsy a strongly vascularized glial tumour was found. The criteria for malignancy according to the WHO classification were only partially fulfilled by this tumour which displayed morphological features of an astrocytoma but could not be furt...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2990.1990.tb00945.x

    authors: Radner H,Kleinert R,Vennigerholz F,Denk H

    更新日期:1990-04-01 00:00:00

  • Cellular and reticular variants of haemangioblastoma revisited: a clinicopathologic study of 88 cases.

    abstract::The presence of histological variants of haemangioblastoma is well established, but data on the prognostic implications of histological subtyping are missing. We thus characterized clinical factors associated with histological subtypes, that is, of the cellular and reticular variant of haemangioblastoma, in a series o...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2990.2005.00669.x

    authors: Hasselblatt M,Jeibmann A,Gerss J,Behrens C,Rama B,Wassmann H,Paulus W

    更新日期:2005-12-01 00:00:00

  • A quantitative assessment of dystrophic mouse (129 ReJ dy/dy) myogenesis in vitro.

    abstract::A quantitative assessment was made of the myogenic capability in vitro of muscle cells from dystrophic (129 Rej dy/dy) and normal mice from birth to 5 months old. Seeding efficiency was increased in dystrophic cells from neonatal and 1-week-old mice compared to age-matched controls. The extent of myogenesis in culture...

    journal_title:Neuropathology and applied neurobiology

    pub_type: 杂志文章

    doi:10.1111/j.1365-2990.1981.tb00098.x

    authors: Summers PJ,Parsons R

    更新日期:1981-07-01 00:00:00