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Retinal Disturbances in Patients and Animal Models with Huntington’s, Parkinson’s and Alzheimer’s Disease

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Abstract

Huntington’s disease (HD), Parkinson’s disease (PD) and Alzheimer’s disease (AD) are neurodegenerative disorders associated with aging. The main hallmarks of these pathologies are deposits of mutant proteins, mitochondrial dysfunctions, reactive oxygen species (ROS) and neuronal death. These diseases affect from heterogeneous form to different brain regions. In fact, psychophysical, electrophysiological and morphological evidence shows that retinal and higher visual centers disturbances occur in HD, PD and AD. Thus, patients with these disorders suffer contrast sensitivity deficits, altered color vision and damaged conscious and unconscious visual perception. These visual deficiencies may contribute to symptoms of these pathologies related to behavior, memory and difficulty in performing daily tasks such as driving, reading or keeping their balance. This chapter tries to integrate the visual changes in pathogenesis and symptomatology of patients with HD, PD and AD and reviews the recent findings in animal models of these diseases to provide an insight into how retinal changes might contribute to symptoms of HD, PD and AD.

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Abbreviations

6-OHDA:

6-Hydroxydopamine

AD:

Alzheimer’s disease

APP:

Amyloid precursor protein

APPswe:

Amyloid precursor protein with the double Swedish mutation

Aβ:

Amyloid β peptides

CS:

Contrast sensitivity

ERG:

Electroretinogram

HD:

Huntington’s disease

htt:

Huntingtin

l-dopa:

l-3,4-Dihydroxyphenylalanine

MPP+:

1-Methyl-4-phenylpyridinium

MPTP:

1-Methyl-4-phenyl-1, 2, 3, 6-tetrahydropyridine

NRSE:

Neuron-restrictive silencer elements

NRSF:

Neuron-restrictive silencer factor

PD:

Parkinson’s disease, PERG, pattern electroretinogram

PINK1:

Phosphatase-tensin homologue induced putative kinase 1

PS1:

Presenilin 1

REST:

Repressor-element 1 silencing transcription factor

ROS:

Reactive oxygen species

VEP:

Visual evoked potentials

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Acknowledgments

We thank Dr. Charles H.V. Hoyle for reading this manuscript and providing useful criticism. This work has been supported by The Comunidad de Madrid NEUROTRANS CM S-SAL-0253-2006.

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  1. Departamento de Bioquímica y Biología Molecular, Escuela de Optica UCM, Madrid, Spain

    C. Santano

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  3. J. Pintor
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  1. Dept. Health & Caring Sciences, University Uppsala, Döbelnsgatan 2, Uppsala, 752 37, Sweden

    Samar Basu

  2. Dept. Surgical Sciences, Anaesthesiology, & Intensive Care, Uppsala University Hospital, Akademiska Sjukhuset, Uppsala, 751 85, Sweden

    Lars Wiklund

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Santano, C., de Lara, M.P., Pintor, J. (2011). Retinal Disturbances in Patients and Animal Models with Huntington’s, Parkinson’s and Alzheimer’s Disease. In: Basu, S., Wiklund, L. (eds) Studies on Experimental Models. Oxidative Stress in Applied Basic Research and Clinical Practice. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-60761-956-7_10

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