Neurofibrillary Tangle

Neurofibrillary Tangle: Pathological clusters of the protein tau that are found within neurons.

Source: RDCRN (NCATS/NIH)1

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Summary

Pathological protein aggregates formed by hyperphosphorylation of a microtubule-associated protein known as tau, causing it to aggregate in an insoluble form. [from HPO]

Source: GTR (NCBI/NIH)2

Introduction: Neurofibrillary Tangle

Neurofibrillary tangles are abnormal accumulations of a protein called tau that collect inside neurons. Healthy neurons, in part, are supported internally by structures called microtubules, which help guide nutrients and molecules from the cell body to the axon and dendrites. In healthy neurons, tau normally binds to and stabilizes microtubules. In Alzheimer’s disease, however, abnormal chemical changes cause tau to detach from microtubules and stick to other tau molecules, forming threads that eventually join to form tangles inside neurons. These tangles block the neuron’s transport system, which harms the synaptic communication between neurons.

Emerging evidence suggests that Alzheimer’s-related brain changes may result from a complex interplay among abnormal tau and beta-amyloid proteins and several other factors. It appears that abnormal tau accumulates in specific brain regions involved in memory. Beta-amyloid clumps into plaques between neurons. As the level of beta-amyloid reaches a tipping point, there is a rapid spread of tau throughout the brain.

Source: NIA (NIH)3

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Neurofibrillary Tangles: Abnormal structures located in various parts of the brain and composed of dense arrays of paired helical filaments (neurofilaments and microtubules). These double helical stacks of transverse subunits are twisted into left-handed ribbon-like filaments that likely incorporate the following proteins: (1) the intermediate filaments: medium- and high-molecular-weight neurofilaments; (2) the microtubule-associated proteins map-2 and tau; (3) actin; and (4) Ubiquitins. As one of the hallmarks of Alzheimer Disease, the neurofibrillary tangles eventually occupy the whole of the cytoplasm in certain classes of cell in the neocortex, hippocampus, brain stem, and diencephalon. The number of these tangles, as seen in post mortem histology, correlates with the degree of dementia during life. Some studies suggest that tangle antigens leak into the systemic circulation both in the course of normal aging and in cases of Alzheimer disease.4

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Neurofibrillary tangles: Pathological protein aggregates formed by hyperphosphorylation of a microtubule-associated protein known as tau, causing it to aggregate in an insoluble form.5

Causes of Neurofibrillary Tangle

Causes may include:6 Causes of Neurofibrillary Tangles:

Terminology for Neurofibrillary Tangle

Synonyms

Neurofibrillary tangles composed of disordered microtubules in neurons ; Paired helical filaments

Source: GTR (NCBI/NIH)7

Categories for Neurofibrillary Tangle

Category of Neurofibrillary tangles:

  • Cerebral inclusion bodies
8

Synonyms and Related Terms

Synonyms of Neurofibrillary tangles:

  • Neurofibrillary tangles composed of disordered microtubules in neurons
  • Paired helical filaments
9

Anatomy Articles

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Disease and Condition Articles

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References

  1. Source: RDCRN (NCATS/NIH): rarediseasesnetwork.org/ cms/ artfl/ Learn-More/ Glossary
  2. Source: GTR (NCBI/NIH): ncbi.nlm.nih.gov/ gtr/ conditions/ CN001981/ 
  3. Source: NIA (NIH): nia.nih.gov/ health/ what-happens-brain-alzheimers-disease
  4. Source: MeSH (U.S. National Library of Medicine)
  5. Source: Human Phenotype Ontology
  6. ibid.
  7. Source: GTR (NCBI/NIH): ncbi.nlm.nih.gov/ gtr/ conditions/ CN001981/ 
  8. Source: Human Phenotype Ontology
  9. ibid.
  10. Source: GTR (NCBI/NIH): ncbi.nlm.nih.gov/ gtr/ conditions/ C1863051/ 

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