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Amyloid Fibrils

Amyloid Fibrils

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,...

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In Vitro Aggregation Assays Using Hyperphosphorylated Tau Protein

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Amyloid Precursor Protein in Abusive Head Trauma Suspects.

Don S Minckler¹, Donald J Brown¹, Angele Nalbandian¹

¹From the Gavin Herbert Eye Institute, Department of Ophthalmology (D.S.M., D.J.B., A.N., D.W.S.), USA; Department of Laboratory Medicine (Ocular Pathology), University of California, Irvine, California, (D.S.M.), USA.

American Journal of Ophthalmology

|March 5, 2022

Summary

Abusive head trauma (AHT) in infants causes axonal transport block in the optic nerve, indicated by amyloid precursor protein (APP-A4) accumulation. This finding supports previous research and highlights the need to monitor intraocular pressure in affected children.

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Induction of Diffuse Axonal Brain Injury in Rats Based on Rotational Acceleration

Induction of Diffuse Axonal Brain Injury in Rats Based on Rotational Acceleration

Published on: May 9, 2020

Area of Science:

Ophthalmology
Neuropathology
Forensic Medicine

Background:

Abusive head trauma (AHT) is a severe form of inflicted traumatic brain injury in infants.
Orthograde axonal transport is crucial for neuronal health, and its disruption can lead to cell death.
Amyloid precursor protein (APP-A4) is a marker of axonal injury and transport deficits.

Purpose of the Study:

To investigate orthograde axonal transport deficits in the retina and optic nerve of suspected abusive head trauma (AHT) cases.
To utilize amyloid precursor protein (APP-A4) immunohistochemistry (IHC) as a biomarker for axonal transport disruption.
To compare findings in AHT suspects with control cases of non-traumatized infants.

Main Methods:

A retrospective case-control study involving 72 eyes from suspected AHT victims and control eyes.
Immunohistochemistry (IHC) for APP-A4 was performed on retinal and optic nerve (lamina cribrosa - LC) tissues.
Quantification of APP-A4 accumulation in the LC was assessed using specialized imaging software in cases with known survival.

Main Results:

Accumulations of APP-A4 were observed in the retinal ganglion cells, nerve fiber layer, and LC axonal bundles of AHT suspects.
94% of AHT suspect eyes with available nerve heads showed positive LC APP-A4 accumulation.
Most AHT cases with documented survival after injury (1-1588 days) exhibited LC APP-A4 accumulation.

Conclusions:

The study confirms orthograde axonal transport block in the lamina cribrosa of children with AHT, as evidenced by APP-A4 IHC.
These findings align with prior research demonstrating similar axonal transport deficits in AHT.
It is recommended to record and manage intraocular pressure in infants diagnosed with or suspected of AHT.