Modeling key pathological features of frontotemporal dementia with C9ORF72 repeat expansion in iPSC-derived human neurons.

abstract：:Progressive supranuclear palsy (PSP) is a 4R-tauopathy predominated by subcortical pathology in neurons, astrocytes, and oligodendroglia associated with various clinical phenotypes. In the present international study, we addressed the question of whether or not sequential distribution patterns can be recognized for PS...

journal_title：Acta neuropathologica

authors： Kovacs GG,Lukic MJ,Irwin DJ,Arzberger T,Respondek G,Lee EB,Coughlin D,Giese A,Grossman M,Kurz C,McMillan CT,Gelpi E,Compta Y,van Swieten JC,Laat LD,Troakes C,Al-Sarraj S,Robinson JL,Roeber S,Xie SX,Lee VM,Trojan

更新日期：2020-08-01 00:00:00

abstract：:Alpha-synuclein (alphaS) and ubiquitin (Ub) are shared constituents of glial cytoplasmic inclusions (GCIs) and Lewy bodies (LBs), both composed of fibrillary structures. Staining profiles of GCIs were investigated with triple immunofluorescence involving immunostaining for alphaS and Ub, both amplified with catalyzed ...

journal_title：Acta neuropathologica

authors： Sakamoto M,Uchihara T,Nakamura A,Mizutani T,Mizusawa H

更新日期：2005-10-01 00:00:00

abstract：:Cerebral β-amyloidosis can be exogenously induced by the intracerebral injection of brain extracts containing aggregated β-amyloid (Aβ) into young, pre-depositing Aβ precursor protein- (APP) transgenic mice. Previous work has shown that the induction involves a prion-like seeding mechanism in which the seeding agent i...

journal_title：Acta neuropathologica

authors： Fritschi SK,Cintron A,Ye L,Mahler J,Bühler A,Baumann F,Neumann M,Nilsson KP,Hammarström P,Walker LC,Jucker M

更新日期：2014-10-01 00:00:00

abstract：:Histochemical and electron microscopic studies were performed in an attempt to clarify the muscle pathology in an 18-year-old man with Fabry disease, showing proximal limb muscle atrophy, and his 52-year-old mother, who is a Fabry carrier with hypertrophic cardiomyopathy. Despite the relatively mild myopathic changes ...

journal_title：Acta neuropathologica

authors： Uchino M,Uyama E,Kawano H,Hokamaki J,Kugiyama K,Murakami Y,Yasue H,Ando M

更新日期：1995-01-01 00:00:00

abstract：:Medulloblastoma (MB) represents approximately 4% of adult brain tumours, and as such is a poorly studied disease. Although many adult MB are treated using paediatric MB protocols, the reported outcomes are inferior to those observed in children. It remains unclear whether biologic differences underlie these clinical o...

journal_title：Acta neuropathologica

authors： Al-Halabi H,Nantel A,Klekner A,Guiot MC,Albrecht S,Hauser P,Garami M,Bognar L,Kavan P,Gerges N,Shirinian M,Roberge D,Muanza T,Jabado N

更新日期：2011-02-01 00:00:00

abstract：:We studied the densities of utrophin and dystrophin at the motor end-plates of patients with myasthenia gravis (MG) using immunohistochemical analysis. The densities were compared with those found in patients with amyotrophic lateral sclerosis, Lambert-Eaton myasthenic syndrome and normal controls. Utrophin was reduce...

journal_title：Acta neuropathologica

authors： Ito H,Yoshimura T,Satoh A,Takino H,Tsujihata M,Nagataki S

更新日期：1996-07-01 00:00:00

abstract：:Massive GGGGCC repeat expansion in the first intron of the gene C9orf72 is the most common known cause of familial frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS). Despite its intronic localization and lack of an ATG start codon, the repeat region is translated in all three reading fra...

journal_title：Acta neuropathologica

authors： Mori K,Arzberger T,Grässer FA,Gijselinck I,May S,Rentzsch K,Weng SM,Schludi MH,van der Zee J,Cruts M,Van Broeckhoven C,Kremmer E,Kretzschmar HA,Haass C,Edbauer D

更新日期：2013-12-01 00:00:00

abstract：:Amyotrophic lateral sclerosis (ALS) is a lethal disease characterized by motor neuron degeneration and associated with aggregation of nuclear RNA-binding proteins (RBPs), including FUS. How FUS aggregation and neurodegeneration are prevented in healthy motor neurons remain critically unanswered questions. Here, we use...

journal_title：Acta neuropathologica

authors： Marrone L,Drexler HCA,Wang J,Tripathi P,Distler T,Heisterkamp P,Anderson EN,Kour S,Moraiti A,Maharana S,Bhatnagar R,Belgard TG,Tripathy V,Kalmbach N,Hosseinzadeh Z,Crippa V,Abo-Rady M,Wegner F,Poletti A,Troost D,A

更新日期：2019-07-01 00:00:00

abstract：:Little information is available regarding the morphological changes in the mitochondria in amyotrophic lateral sclerosis (ALS). In particular, mitochondrial changes in dorsal root ganglion cells have not yet been examined. We therefore conducted an electron microscopic examination of the mitochondria in dorsal root ga...

journal_title：Acta neuropathologica

authors： Sasaki S,Horie Y,Iwata M

更新日期：2007-12-01 00:00:00

abstract：:The cytoskeletal properties and endogenous degradation of intermediate filaments in cultured human glioma cells (U-251MG) were studied using monoclonal antibodies in immunohistochemical and immunochemical methods. Both glial fibrillary acidic protein (GFAP)- and vimentin-antibodies gave a fibrillar cytoplasmic stainin...

journal_title：Acta neuropathologica

authors： Paetau A,Virtanen I

更新日期：1986-01-01 00:00:00

abstract：:In search of novel genes associated with glioma pathogenesis, we have previously shown frequent deletions of the KIAA1797/FOCAD gene in malignant gliomas, and a tumor suppressor function of the encoded focadhesin impacting proliferation and migration of glioma cells in vitro and in vivo. Here, we examined an associati...

journal_title：Acta neuropathologica

authors： Brand F,Förster A,Christians A,Bucher M,Thomé CM,Raab MS,Westphal M,Pietsch T,von Deimling A,Reifenberger G,Claus P,Hentschel B,Weller M,Weber RG

更新日期：2020-01-01 00:00:00

abstract：:Two types of intranuclear inclusions were described in a brain biopsy from a patient with Creutzfeldt-Jakob disease. The first type of intranculear inclusion was papova virus-like and was observed within 20% of the nuclei of all astrocytes and neurons examined. The particles measured 32 nm in diameter and consisted of...

journal_title：Acta neuropathologica

authors： Payne CM,Sibley WA

更新日期：1975-01-01 00:00:00

abstract：:A 1-year-old male chinchilla with a 2-week history of conjunctivitis suffered subsequently from neurological signs comprising seizures, disorientation, recumbency and apathy. After 3 weeks of progressive central nervous disease the animal was killed in view of the poor prognosis. A non-suppurative meningitis and polio...

journal_title：Acta neuropathologica

authors： Wohlsein P,Thiele A,Fehr M,Haas L,Henneicke K,Petzold DR,Baumgärtner W

更新日期：2002-12-01 00:00:00

abstract：:Tumours of the neuroglia, 172 in all (50 glioblastomas, 65 fibrillar and gemistocytic astrocytomas, 26 pilocytic astrocytomas and 31 oligodendrogliomas), were studied by automated microscopic picture analysis. Thirteen morphometric and densitometric parameters of the tumour cell nuclei as well as two mitotic parameter...

journal_title：Acta neuropathologica

authors： Martin H,Voss K

更新日期：1982-01-01 00:00:00

abstract：:A massive expansion of a GGGGCC repeat upstream of the C9orf72 coding region is the most common known cause of amyotrophic lateral sclerosis and frontotemporal dementia. Despite its intronic localization and lack of a canonical start codon, both strands are translated into aggregating dipeptide repeat (DPR) proteins: ...

journal_title：Acta neuropathologica

authors： Schludi MH,May S,Grässer FA,Rentzsch K,Kremmer E,Küpper C,Klopstock T,German Consortium for Frontotemporal Lobar Degeneration.,Bavarian Brain Banking Alliance.,Arzberger T,Edbauer D

更新日期：2015-10-01 00:00:00

abstract：:The conventional concept of Pick's disease does not distinguish Pick's disease with Pick bodies (Pick body disease, PBD) from Pick's disease without Pick bodies [lobar atrophy without Pick bodies, LA-PB(-)]. Recently, intraneuronal ubiquitin-positive inclusions (ub-inclusions), which are thought to be a hallmark of am...

journal_title：Acta neuropathologica

authors： Ikeda K,Akiyama H,Arai T,Ueno H,Tsuchiya K,Kosaka K

更新日期：2002-07-01 00:00:00

abstract：:Abnormal TDP-43 aggregation is a prominent feature in the neuropathology of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration. Mutations in TARDBP, the gene encoding TDP-43, cause some cases of ALS. The normal function of TDP-43 remains incompletely understood. To better understand TDP-43 biolo...

journal_title：Acta neuropathologica

authors： Kraemer BC,Schuck T,Wheeler JM,Robinson LC,Trojanowski JQ,Lee VM,Schellenberg GD

更新日期：2010-04-01 00:00:00

abstract：:We describe the clinical and neuropathological findings of three unrelated autopsy cases of MELAS harboring the A3243G transition in the mitochondrial DNA (mtDNA). Using immunohistochemical techniques, we studied the expression of several subunits of the respiratory chain in various brain regions from the same cases. ...

journal_title：Acta neuropathologica

authors： Sparaco M,Simonati A,Cavallaro T,Bartolomei L,Grauso M,Piscioli F,Morelli L,Rizzuto N

更新日期：2003-09-01 00:00:00

abstract：:DICER1 syndrome is a rare tumor predisposition syndrome with manifestations that predominantly affect children and young adults. The syndrome is typically caused by heterozygous germline loss-of-function DICER1 alterations accompanied on the other allele by somatic missense mutations occurring at one of a few mutation...

journal_title：Acta neuropathologica

authors： de Kock L,Priest JR,Foulkes WD,Alexandrescu S

更新日期：2020-04-01 00:00:00

abstract：:Experimental allergic neuritis was induced in Lewis rats by inoculation with autoreactive T cell lines sensitized to residue 57-81 of P2 myelin protein. Control rats received cells derived from immunization to complete Freund's adjuvant alone. Endoneurial fluid pressure (EFP) was measured in both sciatic nerves at 0, ...

journal_title：Acta neuropathologica

authors： Powell HC,Myers RR,Mizisin AP,Olee T,Brostoff SW

更新日期：1991-01-01 00:00:00

abstract：:The long-term survival of oligodendrocytes in the absence of axons in adult animals was studied following Wallerian degeneration of the optic nerves of adult rats for periods up to 22 months. In contrast to the findings in development and in young animals, large numbers of oligodendrocytes survived during this time pe...

journal_title：Acta neuropathologica

authors： Ludwin SK

更新日期：1990-01-01 00:00:00

abstract：:The diagnosis of infantile neuro-axonal dystrophy (INAD) in a 5-year-old patient was confirmed by the ultrastructural study of neuromuscular, skin and conjunctival biopsy specimens. Abnormal networks of smooth membranous, lamellar and tubular profiles were found in presynaptic terminals and in conjunctival and dermal ...

journal_title：Acta neuropathologica

authors： Martin JJ,Leroy JG,Libert J,van Eygen M,Logghe N

更新日期：1979-03-15 00:00:00

abstract：:The pro-inflammatory and blood-brain barrier (BBB) effects of intratumoral (IT) injection of human recombinant tumor necrosis factor-alpha (rTNF-alpha) were studied in the Fischer rat RT-2 glioma model. Animals received a single stereotaxic injection of either 6 x 10(4) U rTNF-alpha or excipient (vehicle) into the cen...

journal_title：Acta neuropathologica

authors： Wright JL,Merchant RE

更新日期：1997-01-01 00:00:00

abstract：:Motor dysfunction is frequently noted in human immunodeficiency virus type 1 (HIV-1)-infected patients. Until recently, neuropathological changes found in the basal ganglia were advanced as pathogenetic mechanisms. In the present study, further brain structures involved in motor control were analyzed morphometrically....

journal_title：Acta neuropathologica

authors： Abe H,Mehraein P,Weis S

更新日期：1996-08-01 00:00:00

abstract：:A total of 66 skin biopsies from persons with Alzheimer's disease (AD) or Down's syndrome (DS) and from persons without AD were used in this study. The age range was from 7 to 89 years. Positive immunoreactivity of skin biopsies to monoclonal antibody 4G8, which is reactive to amino acid residue 17-24 of synthetic amy...

journal_title：Acta neuropathologica

authors： Wen GY,Wisniewski HM,Blondal H,Benedikz E,Frey H,Pirttila T,Rudelli R,Kim KS

更新日期：1994-01-01 00:00:00

abstract：:Pathways for drainage of interstitial fluid and cerebrospinal fluid from the rat brain were investigated by the injection of 2-5 microliters Indian ink into cerebral white and grey matter and into the subarachnoid space over the vertex of the left frontal lobe. Animals were killed by formalin or glutaraldehyde perfusi...

journal_title：Acta neuropathologica

authors： Zhang ET,Richards HK,Kida S,Weller RO

更新日期：1992-01-01 00:00:00

abstract：:Both protein kinases and phosphoprotein phosphatases are important components of signal transduction systems in cells. Recent studies in Alzheimer's disease (AD) have shown abnormal protein phosphorylation in the cortex suggesting an alteration in these enzymes. In the present study, an antibody against CD45 was used ...

journal_title：Acta neuropathologica

authors： Masliah E,Mallory M,Hansen L,Alford M,Albright T,Terry R,Shapiro P,Sundsmo M,Saitoh T

更新日期：1991-01-01 00:00:00

abstract：:Aluminum was observed in the nucleolus, interchromatin granules, rough endoplasmic reticulum, free ribosomes, euchromatin, and the heterochromatin of the neuron. The association of aluminum with the first four r-RNA-containing cellular components and with the last two DNA-containing chromatins suggests the association...

journal_title：Acta neuropathologica

authors： Wen GY,Wisniewski HM

更新日期：1985-01-01 00:00:00

abstract：:Ubiquitin-immunoreactive (ub-ir) neuronal cytoplasmic inclusions are characteristically found in the extramotor cortex in patients with motor neuron disease and dementia (MND-dementia) and a subset of patients with frontotemporal dementia without motor symptoms (FTD-MND type). Recently, ub-ir neuronal intranuclear inc...

journal_title：Acta neuropathologica

authors： Mackenzie IR,Feldman H

更新日期：2003-06-01 00:00:00

abstract：:Gelatinase A is an enzyme capable of cleaving soluble beta-amyloid protein (beta AP), and may function as an alpha-secretase to produce secretory forms of amyloid precursor protein. We examined gelatinase A immunoreactivity in the brains and posterior roots of neurologically normal, lacunar stroke, Alzheimer disease (...

journal_title：Acta neuropathologica

authors： Yamada T,Miyazaki K,Koshikawa N,Takahashi M,Akatsu H,Yamamoto T

更新日期：1995-01-01 00:00:00