Electroretinography data from ovine models of CLN5 and CLN6 neuronal ceroid lipofuscinoses

被引：6

作者：

Russell, Katharina N. ^{[1
]}

Mitchell, Nadia L. ^{[1
,2
]}

Wellby, Martin P. ^{[1
]}

Barrell, Graham K. ^{[1
]}

Palmer, David N. ^{[1
,2
]}

机构：

[1] Lincoln Univ, Fac Agr & Life Sci, Lincoln, New Zealand

[2] Univ Otago, Dept Radiol, Christchurch, New Zealand

来源：

DATA IN BRIEF | 2021年 / 37卷

关键词：

Neuronal ceroid lipofuscinoses; Electroretinography; Blindness; Sheep; Retinal degeneration;

D O I：

10.1016/j.dib.2021.107188

中图分类号：

O [数理科学和化学]; P [天文学、地球科学]; Q [生物科学]; N [自然科学总论];

学科分类号：

07 ; 0710 ; 09 ;

摘要：

\ This article presents datasets associated with the research article entitled "Intravitreal gene therapy protects against retinal dysfunction and degeneration in sheep with CLN5 Batten disease"(Murray et al., [1]). The neuronal ceroid lipofuscinoses (NCL; Batten disease) are a group of fatal inherited diseases caused by mutations in a number of CLN genes that lead to degenerative and fatal encephalopathies in children. Naturally-occuring sheep models of NCL exist. Affected sheep share the clinical and pathological features of the human disease, including retinal degeneration. Electroretinography (ERG) was employed to characterise the physiological changes in the degenerating retina of CLN5 and CLN6 forms of ovine NCL. ERGs were performed every two months from 3 to 17 months of age in 11 NCL affected (6 CLN5(-/-) and 5 CLN6(-/-)) sheep and 12 clinically normal heterozygous controls (6 CLN5(+/-) and 6 CLN6(+/-)) under three different adaptation conditions. A-wave and b-wave amplitudes were collected from each eye using the Eickemeyer Veterinary ERG system. These are the first longitudinal datasets assessing the progression of retinal degeneration in ovine NCL, aiding in characterisation of the disease process and providing insight into optimal therapeutic windows for subsequent studies. (C) 2021The Authors. Published by Elsevier Inc.

引用

页数：8

共 2 条

[1] Intravitreal gene therapy protects against retinal dysfunction and degeneration in sheep with CLN5 Batten disease [J].

Murray, Samantha J. ;

Russell, Katharina N. ;

Melzer, Tracy R. ;

Gray, Steven J. ;

Heap, Stephen J. ;

Palmer, David N. ;

Mitchell, Nadia L. .

EXPERIMENTAL EYE RESEARCH, 2021, 207

[2]

Russell K., Harvard Dataverse, V1, DOI 10.7910/DVN/JOSWTD

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