Wobbly hedgehog syndrome- a progressive neurodegenerative disease

Grayson A Doss; Daniel Z Radecki; Arya Kethireddy; Madelyn J Reilly; Andrea E Pohly; Benjamin K August; Ian D Duncan; Jayshree Samanta

doi:10.1016/j.expneurol.2023.114520

Wobbly hedgehog syndrome- a progressive neurodegenerative disease

Exp Neurol. 2023 Oct:368:114520. doi: 10.1016/j.expneurol.2023.114520. Epub 2023 Aug 25.

Authors

Grayson A Doss¹, Daniel Z Radecki², Arya Kethireddy², Madelyn J Reilly², Andrea E Pohly³, Benjamin K August⁴, Ian D Duncan⁵, Jayshree Samanta⁶

Affiliations

¹ Department of Surgical Sciences, School of Veterinary Medicine, University of Wisconsin-Madison, 2015 Linden Drive, Madison, WI 53706, USA.
² Department of Comparative Biosciences, School of Veterinary Medicine, University of Wisconsin-Madison, 2015 Linden Drive, Madison, WI 53706, USA.
³ Department of Pathobiological Sciences, School of Veterinary Medicine, University of Wisconsin-Madison, 2015 Linden Drive, Madison, WI 53706, USA.
⁴ University of Wisconsin-Madison, School of Medicine and Public Health, Electron Microscope Facility, USA.
⁵ Department of Medical Sciences, School of Veterinary Medicine, University of Wisconsin-Madison, 2015 Linden Drive, Madison, WI 53706, USA.
⁶ Department of Comparative Biosciences, School of Veterinary Medicine, University of Wisconsin-Madison, 2015 Linden Drive, Madison, WI 53706, USA; Present address: Department of Biomedical Sciences, College of Veterinary Medicine, University of Georgia, 501 DW Brooks Drive, Athens, GA 30602, USA.. Electronic address: jayshree.samanta@uga.edu.

PMID: 37634698
DOI: 10.1016/j.expneurol.2023.114520

Abstract

Wobbly hedgehog syndrome (WHS) has been long considered to be a myelin disease primarily affecting the four-toed hedgehog. In this study, we have shown for the first time that demyelination is accompanied by extensive remyelination in WHS. However, remyelination is not enough to compensate for the axonal degeneration and neuronal loss, resulting in a progressive neurodegenerative disease reminiscent of progressive forms of multiple sclerosis (MS) in humans. Thus, understanding the pathological features of WHS may shed light on the disease progression in progressive MS and ultimately help to develop therapeutic strategies for both diseases.

Keywords: Aberrant myelin; Demyelination; Neurodegeneration; Neuronal cell death; Progressive multiple sclerosis; Remyelination.

Publication types

Research Support, Non-U.S. Gov't
Research Support, N.I.H., Extramural

MeSH terms

Animals
Disease Progression
Hedgehogs
Humans
Memory
Multiple Sclerosis*
Neurodegenerative Diseases* / genetics

Abstract

Publication types

MeSH terms

Grants and funding