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1. GGC repeat expansions in NOTCH2NLC cause uN2CpolyG cerebral amyloid angiopathy NSTL国家科技图书文献中心

Lei,Bao |  Xiaowen,Li... -  《Brain》 - 2025,148(2) - 467~479 - 共13页 - 被引量:2

摘要: lacking inclusions. Congo red-positive amyloid |  staining, ubiquitin, p62 and uN2CpolyG immunostaining on |  frequently in small vessels lacking intranuclear inclusions | Abstract The expansion of GGC repeats within |  NOTCH2NLC leads to translation of the uN2CpolyG protein
关键词: NOTCH2NLC GGC repeat expansions |  neuronal intranuclear inclusion disease |  uN2CpolyG protein |  cerebral amyloid angiopathy |  cerebral microbleeds

2. Dissecting the effect of ALS mutation S375G on the conformational properties and aggregation dynamics of TDP-43370-375 fragment NSTL国家科技图书文献中心

Xu Z. |  Zhang J.... -  《Biophysical Chemistry: An International Journal Devoted to the Physical Chemistry of Biological Phenomena》 - 2024,310 - 共5页 - 被引量:2

摘要: protein of 43 kDa (TDP-43) into ubiquitin-positive |  inclusions is closely associated with amyotrophic lateral | © 2024 Elsevier B.V.The aggregation of |  transactive response deoxyribonucleic acid (DNA) binding |  sclerosis (ALS), frontotemporal lobar degeneration, and
关键词: Aggregation |  Amyotrophic lateral sclerosis |  Molecular dynamics simulation |  Molecular mechanism |  S375G mutation |  TDP-43

3. Defining the Nature and Clinicopathologic Significance of Mallory-Denk-like Inclusions in Ovarian Fibromas: A Potential Degenerative Phenomenon Associated With Torsion NSTL国家科技图书文献中心

Krishnamurthy, Kriti... |  Stillman, Isaac E.... -  《International journal of gynecological pathology: Official journal of the International Society of Gynecological Pathologists》 - 2024,43(3) - 290~295 - 共6页

摘要: without inclusions. The inclusions were positive for |  inclusions are irregularly shaped and eosinophilic as seen |  under the light microscope. MBD-like inclusions have | . Though rare, intracytoplasmic inclusions have been |  inclusions resembling MDBs. Forty-three ovarian fibromas
关键词: Mallory denk bodies |  Cytoplasmic inclusions |  MBD-like inclusions |  Ovarian fibromas |  Type 3 Mallory hyaline |  HEAT-SHOCK PROTEINS |  INTERMEDIATE-FILAMENTS |  BODIES |  DISEASE |  HYALINE...

4. Spinocerebellar ataxia type 4 is caused by a GGC expansion in the ZFHX3 gene and is associated with prominent dysautonomia and motor neuron signs NSTL国家科技图书文献中心

Martin Paucar |  Daniel Nilsson... -  《Journal of Internal Medicine》 - 2024,296(3) - 234~248 - 共15页 - 被引量:3

摘要:, mainly neuronal, inclusions positive for p62 and |  ubiquitin were sparse but widespread in the CNS. This | Abstract Background Spinocerebellar ataxia 4 |  (SCA4), characterized in 1996, features adult‐onset |  ataxia, polyneuropathy, and linkage to chromosome
关键词: nucleotide expansion |  polyglycine |  spinocerebellar ataxia |  ZFHX3

5. Brain DNA methylomic analysis of frontotemporal lobar degeneration reveals OTUD4 in shared dysregulated signatures across pathological subtypes NSTL国家科技图书文献中心

Fodder, Katherine |  Murthy, Megha... -  《Acta Neuropathologica》 - 2023,146(1) - 77~95 - 共19页 - 被引量:2

摘要:-43 positive inclusions (FTLD-TDP) and FTLD with tau | -positive inclusions (FTLD-tau) are the most common |  were enriched for genes implicated in the ubiquitin | Frontotemporal lobar degeneration (FTLD) is an |  umbrella term describing the neuropathology of a
关键词: DNA methylation |  Frontotemporal dementia |  Progressive supranuclear palsy |  Human brain tissue |  EWAS |  Co-methylation |  PROGRESSIVE SUPRANUCLEAR PALSY |  STRESS GRANULES |  PROTEIN |  METHYLATION...

6. The ovine Type II Gaucher disease model recapitulates aspects of human brain disease. NSTL国家科技图书文献中心

Winner, Leanne K. |  Beard, Helen... -  《Biochimica et biophysica acta. Molecular basis of disease: BBA》 - 2023,1869(4) - 共0页 - 被引量:1

摘要: inclusions reactive for TOMM20, ubiquitin and most |  shrunken and deeply Nissl stain positive. Amoeboid | Acute neuronopathic (type II) Gaucher disease |  (GD) is a devastating, untreatable neurological |  disorder resulting from mutations in the
关键词: Sheep |  Lysosomal storage disorder |  Neuronopathic |  Gliosis |  Apoptosis |  Sphingolipidoses |  MOUSE MODEL |  GLUCOCEREBROSIDASE |  DEFICIENCY |  ICHTHYOSIS...

7. Stress-inducible phosphoprotein 1 (HOP/STI1/STIP1) regulates the accumulation and toxicity of α-synuclein in vivo SCIE Web of Science核心 NSTL国家科技图书文献中心

Rachel E.,Lackie |  Aline S.,de Miranda... -  《Acta Neuropathologica》 - 2022,144(5) - 881~910 - 共30页 - 被引量:8

摘要: phosphorylated α-synuclein in ubiquitin-positive inclusions. In |  accompanied by inclusions when injected with α-synuclein pre | Abstract The predominantly pre-synaptic |  intrinsically disordered protein α-synuclein is prone to |  misfolding and aggregation in synucleinopathies, such as
关键词: Chaperone |  Hsp90 |  Hsp70 |  STIP1 |  HOP |  Parkinson’s |  Lewy body |  Touchscreens |  α-Synuclein |  Neuropathology...

8. Cytoplasmic TDP-43 is involved in cell fate during stress recovery SCIE Web of Science核心 NSTL国家科技图书文献中心

Lee, Youn-Bok |  Scotter, Emma L.... -  《Human Molecular Genetics》 - 2022,31(2) - 166~175 - 共10页 - 被引量:9

摘要:-positive inclusions in affected neurons and glia |  patients to the cytoplasm, where it forms ubiquitin |  TIA-1 positive SGs. We demonstrate that inducing | Transactive response DNA binding protein 43 |  (TDP-43) is an RNA processing protein central to the
关键词: AMYOTROPHIC-LATERAL-SCLEROSIS |  DNA-BINDING PROTEIN |  SPLICING REGULATION |  ALZHEIMERS-DISEASE |  GRANULES |  FUS |  LOCALIZATION |  MUTATIONS |  DEMENTIA

9. Biophysical characterization of full‐length TAR DNA‐binding protein (TDP‐43) phase separation NSTL国家科技图书文献中心

Tommaso Staderini |  Alessandra Bigi... -  《Protein science :》 - 2022,31(12) - e4509~e4509 - 共16页 - 被引量:8

摘要: frontotemporal lobar degeneration with ubiquitinpositive |  inclusions are associated with deposition of cytosolic | Abstract Amyotrophic lateral sclerosis and |  inclusion bodies of TAR DNA‐binding protein 43 (TDP‐43) in |  brain and motor neurons. We induced phase separation
关键词: ALS |  FTLD |  liquid–liquid phase separation |  motor neuron diseases |  recombinant TDP‐43 |  self‐assembly

10. The clinical and neuropathological picture of adult neuronal intranuclear inclusion disease with no radiological abnormality NSTL国家科技图书文献中心

Miki, Yasuo |  Kamata, Kosuke... -  《Neuropathology :》 - 2022,42(3) - 204~211 - 共8页 - 被引量:3

摘要:, ubiquitin-, p62- and uN2CpolyG-positive neuronal |  glial cells harbor intranuclear inclusions. In |  addition, these inclusions are present in the peripheral |  intranuclear inclusions were particularly frequent in the |  intranuclear inclusions were barely evident in the affected
关键词: heterogeneity |  immunoprofile |  intranuclear inclusions |  neuronal intranuclear inclusion disease |  NOTCH2NLC |  NUCLEAR INCLUSIONS |  POLYGLUTAMINE DISEASES |  BODY DISEASE |  HYALINE INCLUSIONS |  IMMUNOREACTIVITY...
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