Inclusion-body myositis: newest concepts of pathogenesis and relation to aging and Alzheimer disease.

Abstract:

:We review the newest advances related to seeking the pathogenic mechanism(s) of sporadic inclusion-body myositis (s-IBM) and present the pathologic diagnostic criteria of s-IBM. We discuss the possible pathogenic role of several themes, such as 1) increased amyloid-beta precursor protein (AbetaPP) and of its fragment Abeta; 2) phosphorylation of tau protein; 3) oxidative stress; 4) abnormal a) signal-transduction, b) transcription, and c) RNA accumulation; 5) "junctionalization" and myogenous" denervation; and 6) lymphocytic inflammation. Evidence is provided supporting our hypothesis that overexpression of AbetaPP within the aging muscle fibers is an early upstream event causing the subsequent pathogenic cascade. The remarkable pathologic similarities between s-IBM muscle and Alzheimer disease (AD) brain are discussed, and the possible cause and significance are addressed.

authors

Askanas V,Engel WK

doi

10.1093/jnen/60.1.1

subject

Has Abstract

pub_date

2001-01-01 00:00:00

pages

1-14

issue

1

eissn

0022-3069

issn

1554-6578

journal_volume

60

pub_type

杂志文章,评审
  • Traumatically induced reactive change as visualized through the use of monoclonal antibodies targeted to neurofilament subunits.

    abstract::Reactive axonal change has long been recognized as a feature of traumatic brain injury. To date, the histological methods used to identify reactive axons have been of limited utility, and they have not provided insight into the initial intraaxonal event that triggers reactive change. In this investigation, monoclonal ...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章

    doi:10.1097/00005072-199203000-00006

    authors: Yaghmai A,Povlishock J

    更新日期:1992-03-01 00:00:00

  • First report of Creutzfeldt-Jakob disease occurring in 2 siblings unexplained by PRNP mutation.

    abstract::Sibling concurrence of pathologically confirmed prion disease has only been reported in association with pathogenic mutation of the prion protein gene (PRNP). Here, we report 2 siblings with classic neuropathologic features of sporadic Creutzfeldt-Jakob disease unexplained by PRNP mutation or known risk factors for ia...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章

    doi:10.1097/NEN.0b013e318182f36e

    authors: Webb TE,Pal S,Siddique D,Heaney DC,Linehan JM,Wadsworth JD,Joiner S,Beck J,Wroe SJ,Stevenson V,Brandner S,Mead S,Collinge J

    更新日期:2008-09-01 00:00:00

  • Ultrastructural analysis and TUNEL demonstrate motor neuron apoptosis in Werdnig-Hoffmann disease.

    abstract::Werdnig-Hoffmann disease (WHD) is the most severe clinical type of spinal muscular atrophy characterized by loss of lower motor neurons and paralysis. We examined the hypothesis that disease pathogenesis is based on an inappropriate persistence of normally occurring motor neuron programmed cell death. The diagnosis of...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章

    doi:10.1093/jnen/59.5.398

    authors: Simic G,Seso-Simic D,Lucassen PJ,Islam A,Krsnik Z,Cviko A,Jelasic D,Barisic N,Winblad B,Kostovic I,Kruslin B

    更新日期:2000-05-01 00:00:00

  • Immunization with neurofilament light protein induces spastic paresis and axonal degeneration in Biozzi ABH mice.

    abstract::Axonal damage is the major cause of irreversible neurologic disability in patients with multiple sclerosis. Although axonal damage correlates with antibodies against neurofilament light (NF-L) protein, a major component of the axonal cytoskeleton, the possible pathogenic role of autoimmunity to axonal antigens such as...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章

    doi:10.1097/nen.0b013e318040ad5c

    authors: Huizinga R,Heijmans N,Schubert P,Gschmeissner S,'t Hart BA,Herrmann H,Amor S

    更新日期:2007-04-01 00:00:00

  • Increased hippocampal AMPA and NMDA receptor subunit immunoreactivity in temporal lobe epilepsy patients.

    abstract::This study determined if hippocampal AMPA and NMDA subunit immunoreactivity (IR) in temporal lobe epilepsy patients was increased compared with nonseizure autopsies. Hippocampi from hippocampal sclerosis patients (HS; n = 26) and nonsclerosis cases (non-HS: n = 12) were compared with autopsies (n = 6) and studied for ...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章

    doi:10.1097/00005072-199806000-00008

    authors: Mathern GW,Pretorius JK,Mendoza D,Lozada A,Leite JP,Chimelli L,Fried I,Sakamoto AC,Assirati JA,Adelson PD

    更新日期:1998-06-01 00:00:00

  • Cytokines and Mycobacterium leprae induce apoptosis in human Schwann cells.

    abstract::The development of deformities during the course of leprosy disease is a major public health concern worldwide. It is possible that cytokine production and apoptosis of Schwann cells (SCs) directly affect nerve degeneration and regeneration leading to injury of the myelin sheath and axon. In the present study, the exp...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章

    doi:10.1097/01.jnen.0000182982.09978.66

    authors: Oliveira RB,Sampaio EP,Aarestrup F,Teles RM,Silva TP,Oliveira AL,Antas PR,Sarno EN

    更新日期:2005-10-01 00:00:00

  • The Serotonin Brainstem Hypothesis for the Sudden Infant Death Syndrome.

    abstract::The sudden infant death syndrome (SIDS) is the leading cause of postneonatal infant mortality in the United States today, with an overall rate of 0.39/1000 live births. It is defined as the sudden and unexpected death of an infant <12 months of age that remains unexplained after a complete autopsy, death scene investi...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章,评审

    doi:10.1093/jnen/nlz062

    authors: Kinney HC,Haynes RL

    更新日期:2019-09-01 00:00:00

  • Axotomized motoneurons can be rescued from cell death by peripheral nerve grafts: the effect of donor age.

    abstract::Injury to neonatal nerves, unlike adult nerves, results in poor regeneration and extensive motoneuron death. We examined whether exposure to a more mature nerve environment could rescue axotomized motoneurons following neonatal injury. The sciatic nerve in 1 hindlimb of 3-day-old (P3) rats was transected and the cut e...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章

    doi:10.1093/jnen/62.1.75

    authors: Low HL,Nogradi A,Vrbová G,Greensmith L

    更新日期:2003-01-01 00:00:00

  • Neuropathology of herpes simplex virus encephalitis in a rat seizure model.

    abstract::Herpes simplex virus type 1 (HSV-1) is the cause of a serious and often fatal encephalitis. Patients who survive herpes simplex encephalitis (HSE) experience behavioral abnormalities including profound cognitive dysfunctions. We have developed a rat model of acute HSE to investigate the cognitive impairments caused by...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章

    doi:10.1097/00005072-199305000-00008

    authors: Beers DR,Henkel JS,Schaefer DC,Rose JW,Stroop WG

    更新日期:1993-05-01 00:00:00

  • Phenotypic differences between human monocytes/macrophages and microglial cells studied in situ and in vitro.

    abstract::This report describes a phenotypic differentiation pattern conceived to distinguish invading monocytes from resident microglia in frozen and formalin-fixed human CNS. Phagocytic cells in normal and diseased CNS (multiple sclerosis and encephalitis) were studied immunohistochemically with a panel of antibodies, and phe...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章

    doi:10.1097/00005072-199409000-00008

    authors: Ulvestad E,Williams K,Mørk S,Antel J,Nyland H

    更新日期:1994-09-01 00:00:00

  • LR11/SorLA expression is reduced in sporadic Alzheimer disease but not in familial Alzheimer disease.

    abstract::LR11 is an ApoE receptor that is enriched in the brain. We have shown that LR11 is markedly downregulated in patients with sporadic Alzheimer disease (AD). This finding led us to explore whether reduced LR11 expression reflects a primary mechanism of disease or merely a secondary consequence of other AD-associated cha...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章

    doi:10.1097/01.jnen.0000228205.19915.20

    authors: Dodson SE,Gearing M,Lippa CF,Montine TJ,Levey AI,Lah JJ

    更新日期:2006-09-01 00:00:00

  • Analysis of hemangiopericytic meningiomas by immunohistochemistry, electron microscopy and cell culture.

    abstract::Intracranial hemangiopericytic meningiomas (HM) from seven patients were examined by immunostaining, transmission electron microscopy (TEM), scanning electron microscopy (SEM) and cell culture. Positive staining for Factor VIII-related antigen was restricted to capillary endothelial cells. There was no reaction with a...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章

    doi:10.1097/00005072-198701000-00006

    authors: Nakamura M,Inoue HK,Ono N,Kunimine H,Tamada J

    更新日期:1987-01-01 00:00:00

  • miR-30d-5p Plays an Important Role in Autophagy and Apoptosis in Developing Rat Brains After Hypoxic-Ischemic Injury.

    abstract::Increasing evidence has demonstrated a vital role of microRNAs (miRNAs) in diverse biological processes. However, their functions in developing brain with hypoxia-ischemia (HI) remain largely unknown. Through a miRNA microarray analysis in a P10 rat model of cerebral HI, we found that miR-30d-5p was one of the most de...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章

    doi:10.1093/jnen/nlx052

    authors: Zhao F,Qu Y,Zhu J,Zhang L,Huang L,Liu H,Li S,Mu D

    更新日期:2017-08-01 00:00:00

  • Mechanisms of axonal spheroid formation in central nervous system Wallerian degeneration.

    abstract::Wallerian degeneration of the CNS is accompanied by axonal dystrophy or swelling. To understand the mechanisms by which swellings arise, we studied their spatiotemporal dynamics, ultrastructure, composition, and the conditions that affect their formation in vivo and ex vivo. In contrast to peripheral nerve axons, lesi...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章

    doi:10.1097/NEN.0b013e3181da84db

    authors: Beirowski B,Nógrádi A,Babetto E,Garcia-Alias G,Coleman MP

    更新日期:2010-05-01 00:00:00

  • Three-dimensional analysis of the relationship between synaptic pathology and neuropil threads in Alzheimer disease.

    abstract::Recent studies have shown that the Alzheimer disease (AD) neocortex is characterized by a loss of large neurons, the presence of dilated terminal axons, widespread loss of synapses, and a disruption of the dendritic cytoskeleton which is manifested as Tau immunoreactive threads. In the present study we have investigat...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章

    doi:10.1097/00005072-199207000-00003

    authors: Masliah E,Ellisman M,Carragher B,Mallory M,Young S,Hansen L,DeTeresa R,Terry RD

    更新日期:1992-07-01 00:00:00

  • Peri-Infarct Upregulation of the Oxytocin Receptor in Vascular Dementia.

    abstract::Vascular dementia (VaD) is cognitive decline linked to reduced cerebral blood perfusion, yet there are few therapeutic options to protect cognitive function following cerebrovascular accidents. The purpose of this study was to profile gene expression changes unique to VaD to identify and characterize disease relevant ...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章

    doi:10.1093/jnen/nlz023

    authors: McKay EC,Beck JS,Khoo SK,Dykema KJ,Cottingham SL,Winn ME,Paulson HL,Lieberman AP,Counts SE

    更新日期:2019-05-01 00:00:00

  • Lewy body pathology in normal elderly subjects.

    abstract::Lewy bodies and Lewy neurites are the hallmark neuropathologic findings in Parkinson disease, Parkinson disease with dementia, dementia with Lewy bodies, and other alpha-synucleinopathies. They have also been described in the brains of normal older individuals and referred to as incidental Lewy body disease. The purpo...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章

    doi:10.1097/NEN.0b013e3181ac10a7

    authors: Markesbery WR,Jicha GA,Liu H,Schmitt FA

    更新日期:2009-07-01 00:00:00

  • Cytogenetic, molecular genetic and pathological analyses in 126 meningiomas.

    abstract::In a series of 126 meningiomas, tumor and patient characteristics were investigated and statistically analyzed. A combined cytogenetic and molecular genetic approach was used to study chromosomal abnormalities and loss of markers on chromosome 22q. This approach was successfully applied to 93 meningiomas. In 66 cases,...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章

    doi:10.1097/00005072-199503000-00009

    authors: Lekanne Deprez RH,Riegman PH,van Drunen E,Warringa UL,Groen NA,Stefanko SZ,Koper JW,Avezaat CJ,Mulder PG,Zwarthoff EC

    更新日期:1995-03-01 00:00:00

  • Mild cognitive impairment and asymptomatic Alzheimer disease subjects: equivalent β-amyloid and tau loads with divergent cognitive outcomes.

    abstract::Older adults with intact cognition before death and substantial Alzheimer disease (AD) lesions at autopsy have been termed "asymptomatic AD subjects" (ASYMAD). We previously reported hypertrophy of neuronal cell bodies, nuclei, and nucleoli in the CA1 of the hippocampus (CA1), anterior cingulate gyrus, posterior cingu...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章

    doi:10.1097/NEN.0000000000000052

    authors: Iacono D,Resnick SM,O'Brien R,Zonderman AB,An Y,Pletnikova O,Rudow G,Crain B,Troncoso JC

    更新日期:2014-04-01 00:00:00

  • Infiltrating Hematogenous Macrophages Aggregate Around β-Amyloid Plaques in an Age- and Sex-Dependent Manner in a Mouse Model of Alzheimer Disease.

    abstract::β-Amyloid (Aβ) plaques can trigger chronic inflammation in the cellular environment that recruits infiltrating macrophages during the course of Alzheimer disease (AD). Activated macrophages release pro-inflammatory cytokines that increase neurotoxicity associated with AD. A major impediment to investigating neuroinfla...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章

    doi:10.1093/jnen/nlaa093

    authors: Kozyrev N,Albers S,Yang J,Prado VF,Prado MAM,Fonseca GJ,Rylett RJ,Dekaban GA

    更新日期:2020-11-01 00:00:00

  • Histopathologic Analysis of Cerebral Autosomal Recessive Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CARASIL): A Report of a New Genetically Confirmed Case and Comparison to 2 Previous Cases.

    abstract::Cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy (CARASIL) is a nonhypertensive hereditary cerebral small vessel disease that is caused by mutations in a single gene, HTRA1. The HTRA1 protein normally represses transforming growth factor-β (TGF-β) signaling and its mutations ...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章

    doi:10.1093/jnen/nlw078

    authors: Ito S,Takao M,Fukutake T,Hatsuta H,Funabe S,Ito N,Shimoe Y,Niki T,Nakano I,Fukayama M,Murayama S

    更新日期:2016-11-01 00:00:00

  • Arterial wall degeneration plus hemodynamic insult cause arterial wall remodeling and nascent aneurysm formation at specific sites in dogs.

    abstract::To determine whether arterial wall degeneration, in combination with hemodynamic insult, causes cerebral artery aneurysms in a dog model, we simulated the geometry and hemodynamics of a human artery by surgical reconstruction of both common carotid arteries in 12 dogs. The dogs were then randomly assigned to one of th...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章

    doi:10.1097/NEN.0000000000000100

    authors: Zhu YQ,Li MH,Yan L,Tan HQ,Cheng YS

    更新日期:2014-09-01 00:00:00

  • Covalent crosslinking of neurofilament proteins by oxidized catechols as a potential mechanism of Lewy body formation.

    abstract::Brainstem Lewy bodies (LB) are neuronal inclusions that are closely related to Parkinson's disease (PD). The filamentous component of LB from patients with PD contains biochemically altered neurofilaments (NF). Herein we have tested the hypothesis that the oxidized products of catechols may covalently crosslink NF. Ne...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章

    doi:10.1097/00005072-199505000-00004

    authors: Montine TJ,Farris DB,Graham DG

    更新日期:1995-05-01 00:00:00

  • Posttreatment Effect of MGMT Methylation Level on Glioblastoma Survival.

    abstract::The DNA repair protein O6-methylguanine-DNA methyltransferase (MGMT) removes temozolomide-induced alkylation, thereby preventing DNA damage and cytotoxicity. We investigated the prognostic effect of different MGMT methylation levels on overall and progression-free survival in 327 patients with primary glioblastoma und...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章

    doi:10.1093/jnen/nlz032

    authors: Dahlrot RH,Larsen P,Boldt HB,Kreutzfeldt MS,Hansen S,Hjelmborg JB,Kristensen BW

    更新日期:2019-07-01 00:00:00

  • The axonal pathology in chronic IDPN intoxication.

    abstract::Neurofilamentous axonal swellings occur in a number of degenerative and toxic disorders of the nervous system. In one of these, experimental intoxication with beta, beta'-iminodiproprionitrile (IDPN), accumulation of neurofilaments has been shown to result from a defect in slow axonal transport. The consequence of thi...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章

    doi:10.1097/00005072-198001000-00004

    authors: Clark AW,Griffin JW,Price DL

    更新日期:1980-01-01 00:00:00

  • Synaptic ultrastructural alterations anticipate the development of neuroaxonal dystrophy in sympathetic ganglia of aged and diabetic mice.

    abstract::Neuroaxonal dystrophy, a distinctive axonopathy characterized by marked enlargement of distal axons, is the hallmark pathologic alteration in aged and diabetic human prevertebral sympathetic ganglia and in corresponding rodent models. Neuroaxonal dystrophy is thought to represent the abnormal outcome of cycles of syna...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章

    doi:10.1097/NEN.0b013e318190d6db

    authors: Schmidt RE,Parvin CA,Green KG

    更新日期:2008-12-01 00:00:00

  • Distinct underlying mechanisms of limb and respiratory muscle fiber weaknesses in nemaline myopathy.

    abstract::Nemaline myopathy is the most common congenital myopathy and is caused by mutations in various genes such as ACTA1 (encoding skeletal α-actin). It is associated with limb and respiratory muscle weakness. Despite increasing clinical and scientific interest, the molecular and cellular events leading to such weakness rem...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章

    doi:10.1097/NEN.0b013e318293b1cc

    authors: Lindqvist J,Cheng AJ,Renaud G,Hardeman EC,Ochala J

    更新日期:2013-06-01 00:00:00

  • Fine structure of the cerebellum of "staggerer-reeler", a double mutant of mice affected by staggerer and reeler conditions. III. bergmann fiber anomalies.

    abstract::Bergmann fibers and the distribution of Golgi epithelial cells were significantly altered in staggerer, reeler and double mutant (affected by both staggerer and reeler conditions). However, the Bergmann fiber anomalies in double mutant were not identical to those of either staggerer or reeler. A comparison of these an...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章

    doi:10.1097/00005072-197705000-00003

    authors: Yoon CH

    更新日期:1977-05-01 00:00:00

  • Reduced expression of the P2 form of the gap junction protein connexin43 in malignant meningiomas.

    abstract::Neoplastic transformation is often associated with aberrant gap junctional intercellular communication. We assessed mutations and expression of the connexin43 (Cx43) gene in 49 intracranial meningiomas. SSCP analyses followed by direct DNA sequencing showed GCG-->GTG (Ala-->Val) transition mutation in codon 253 of the...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章

    doi:

    authors: Sato K,Gratas C,Lampe J,Biernat W,Kleihues P,Yamasaki H,Ohgaki H

    更新日期:1997-07-01 00:00:00

  • Progression of cerebral amyloid angiopathy in transgenic mouse models of Alzheimer disease.

    abstract::Cerebral amyloid angiopathy (CAA), the deposition of beta-amyloid (Abeta3) in cerebral vessels, has been implicated as a common cause of hemorrhagic stroke and other forms of vascular disease. CAA is also a frequent concomitant of Alzheimer disease (AD). While the longterm consequences of CAA are well recognized from ...

    journal_title:Journal of neuropathology and experimental neurology

    pub_type: 杂志文章

    doi:10.1097/01.jnen.0000171644.00180.fc

    authors: Domnitz SB,Robbins EM,Hoang AW,Garcia-Alloza M,Hyman BT,Rebeck GW,Greenberg SM,Bacskai BJ,Frosch MP

    更新日期:2005-07-01 00:00:00