Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Concept Videos

Amyloid Fibrils03:03

Amyloid Fibrils

12.9K
Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining,...
12.9K
Amyloid Fibrils03:03

Amyloid Fibrils

6.9K
6.9K

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Skin-limited arteritis of small muscular arteries with giant cell-rich granulomatous inflammation in a patient with polymyalgia rheumatica.

Scandinavian journal of rheumatology·2018
Same author

Body weight and dysautonomia in early Parkinson's disease.

Acta neurologica Scandinavica·2016
Same author

Therapeutic implication of genetic variants of IL13 and STAT4 in airway remodelling with bronchial asthma.

Clinical and experimental allergy : journal of the British Society for Allergy and Clinical Immunology·2016
Same author

Increased Facet Fluid Predicts Dynamic Changes in the Dural Sac Size on Axial-Loaded MRI in Patients with Lumbar Spinal Canal Stenosis.

AJNR. American journal of neuroradiology·2015
Same author

Downregulation of miRNA-31 induces taxane resistance in ovarian cancer cells through increase of receptor tyrosine kinase MET.

Oncogenesis·2013
Same author

Application of 62Cu-diacetyl-bis (N4-methylthiosemicarbazone) PET imaging to predict highly malignant tumor grades and hypoxia-inducible factor-1α expression in patients with glioma.

AJNR. American journal of neuroradiology·2012

Related Experiment Video

Updated: Mar 26, 2026

Evaluation of LC3-II Release via Extracellular Vesicles in Relation to the Accumulation of Intracellular LC3-positive Vesicles
06:58

Evaluation of LC3-II Release via Extracellular Vesicles in Relation to the Accumulation of Intracellular LC3-positive Vesicles

Published on: October 18, 2024

1.4K

Axonal TDP-43 aggregates in sporadic amyotrophic lateral sclerosis.

T Onozato1,2, A Nakahara1, E Suzuki-Kouyama1

  • 1Division of Neuropathology, Department of Brain Disease Research, Shinshu University School of Medicine, Nagano, Japan.

Neuropathology and Applied Neurobiology
|January 29, 2016
PubMed
Summary

Axonal aggregates of phosphorylated TDP-43 (p-TDP-43) in sporadic ALS were studied. Two types of aggregates, MAs and GRAs, were found in motor neurons and may shorten disease course.

Keywords:
amyotrophic lateral sclerosisaxonal aggregatesynapsetopographytransactive response DNA-binding protein 43 kDa

More Related Videos

Optogenetic Phase Transition of TDP-43 in Spinal Motor Neurons of Zebrafish Larvae
07:14

Optogenetic Phase Transition of TDP-43 in Spinal Motor Neurons of Zebrafish Larvae

Published on: February 25, 2022

6.6K
Novel Atomic Force Microscopy Based Biopanning for Isolation of Morphology Specific Reagents against TDP-43 Variants in Amyotrophic Lateral Sclerosis
13:31

Novel Atomic Force Microscopy Based Biopanning for Isolation of Morphology Specific Reagents against TDP-43 Variants in Amyotrophic Lateral Sclerosis

Published on: February 12, 2015

9.3K

Related Experiment Videos

Last Updated: Mar 26, 2026

Evaluation of LC3-II Release via Extracellular Vesicles in Relation to the Accumulation of Intracellular LC3-positive Vesicles
06:58

Evaluation of LC3-II Release via Extracellular Vesicles in Relation to the Accumulation of Intracellular LC3-positive Vesicles

Published on: October 18, 2024

1.4K
Optogenetic Phase Transition of TDP-43 in Spinal Motor Neurons of Zebrafish Larvae
07:14

Optogenetic Phase Transition of TDP-43 in Spinal Motor Neurons of Zebrafish Larvae

Published on: February 25, 2022

6.6K
Novel Atomic Force Microscopy Based Biopanning for Isolation of Morphology Specific Reagents against TDP-43 Variants in Amyotrophic Lateral Sclerosis
13:31

Novel Atomic Force Microscopy Based Biopanning for Isolation of Morphology Specific Reagents against TDP-43 Variants in Amyotrophic Lateral Sclerosis

Published on: February 12, 2015

9.3K

Area of Science:

  • Neuroscience
  • Pathology
  • Biochemistry

Background:

  • Sporadic amyotrophic lateral sclerosis (sALS) is a neurodegenerative disease characterized by motor neuron loss.
  • Phosphorylated transactive response DNA-binding protein 43 kDa (p-TDP-43) aggregates are a hallmark pathology in sALS.
  • Understanding the distribution and characteristics of these aggregates is crucial for elucidating disease mechanisms.

Purpose of the Study:

  • To investigate the presence and topographical distribution of axonal aggregates of p-TDP-43 in Japanese patients with sALS.
  • To characterize the microscopic features of these aggregates and their relationship with neurofilaments.
  • To correlate the presence of specific aggregate types with the clinical course of sALS.

Main Methods:

  • Immunohistochemical examination of brain and spinal cord specimens from sALS patients and controls.
  • Analysis of formalin-fixed paraffin-embedded tissues.
  • Assessment of topographical distribution, microscopic features (dash-like granuloreticular aggregates - GRAs, massive aggregates - MAs), presynaptic aggregates, and correlation with clinical data.

Main Results:

  • p-TDP-43 aggregates (GRAs and MAs) were frequently observed in axons of hypoglossal and facial nerve fibers, and spinal anterior horn cells.
  • MAs were associated with surrounding phosphorylated neurofilaments, while GRAs showed decreased p-neurofilament immunoreactivity within axons.
  • Patients with MAs and GRAs exhibited a shorter clinical course compared to those without these aggregates.
  • Neuronal loss of nuclear TDP-43 and presence of cytoplasmic inclusions were noted in the red nucleus, with presynaptic TDP-43 aggregates potentially affecting postsynaptic neuronal nuclei.

Conclusions:

  • Two distinct types of axonal p-TDP-43 aggregates, MAs and GRAs, are predominantly found in motor pathways (facial and hypoglossal nuclei, anterior horn cells) in sALS.
  • These aggregates may play a role in neuronal dysfunction and disease progression.
  • Presynaptic TDP-43 aggregates appear to induce the loss of TDP-43 in postsynaptic neuronal nuclei.