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 Experiment Videos

Intermitochondrial septate structures in dystrophic axons

P L Ramos, K Wisniewski, G A Jervis

    Acta Neuropathologica
    |January 1, 1980
    PubMed
    Summary
    This summary is machine-generated.

    Related Concept Videos

    You might also read

    Related Articles

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

    Sort by
    Same author

    Studies on phenylpyruvic oligophrenia; the position of the metabolic error.

    The Journal of biological chemistry·2010
    Same author

    Increased plasma amyloid beta protein 1-42 levels in Down syndrome.

    Neuroscience letters·1998
    Same author

    A novel Polish presenilin-1 mutation (P117L) is associated with familial Alzheimer's disease and leads to death as early as the age of 28 years.

    Neuroreport·1998
    Same author

    N-methyl-D-aspartate receptor-mediated, calcium-induced calcium release in rat dentate gyrus/CA4 in vivo.

    Journal of neuroscience research·1998
    Same author

    Human cystatin C forms an inactive dimer during intracellular trafficking in transfected CHO cells.

    Journal of cellular physiology·1997
    Same author

    Amyloid angiopathy and blood-brain barrier changes in Alzheimer's disease.

    Annals of the New York Academy of Sciences·1997
    Same journal

    Annexin A11 and TDP-43: core players in neurodegeneration.

    Acta neuropathologica·2026
    Same journal

    CTE-type tau filaments in Alzheimer's disease with co-morbid LATE-NC.

    Acta neuropathologica·2026
    Same journal

    Mutation-specific neuropathologic signatures in MAPT-associated frontotemporal lobar degeneration.

    Acta neuropathologica·2026
    Same journal

    Molecular changes during AT/RT progression associated with epithelial-mesenchymal transition and extracellular matrix changes.

    Acta neuropathologica·2026
    Same journal

    Prion-like transmission and propagation of human β-amyloid to the bank vole rodent model.

    Acta neuropathologica·2026
    Same journal

    Inhibition of tRNA fragments dysregulated in human mTLE exacerbates pathology and seizure activity.

    Acta neuropathologica·2026
    See all related articles

    Novel intermitochondrial septate structures were identified in infantile neuroaxonal dystrophy axons. These structures, previously unseen in the human central nervous system (CNS), represent complex mitochondrial membrane interdigitation, not true junctions.

    Area of Science:

    • Neuroscience
    • Cell Biology
    • Pathology

    Background:

    • Infantile neuroaxonal dystrophy (INAD) is a rare genetic disorder affecting the nervous system.
    • Septate structures have been observed in various tissues and tumors but not previously in the human central nervous system (CNS).

    Purpose of the Study:

    • To investigate the nature of unique intermitochondrial septate structures found in dystrophic axons in INAD.
    • To determine if these structures represent a novel pathological feature in human CNS disorders.

    Main Methods:

    • Transmission electron microscopy (TEM) and transmission tilt electron microscopy (TTEM) were employed.
    • Structural analysis, model construction, and X-ray diffraction were utilized.
    • Detailed ultrastructural examination of affected axons.

    Related Experiment Videos

    Main Results:

    • Intermitochondrial septate structures were consistently observed in dystrophic axons of INAD patients.
    • These structures exhibit a periodicity of 120 Å, consistent with previously reported similar structures.
    • Ultrastructural analysis revealed the structures are complex interdigitations of outer mitochondrial membranes, not true intercellular junctions.

    Conclusions:

    • The identified intermitochondrial septate structures are a novel finding within the human CNS, specifically in infantile neuroaxonal dystrophy.
    • These structures are characterized by complex mitochondrial membrane interdigitation, differing from true junctions.
    • Further research is warranted to understand the formation and functional implications of these structures in INAD.