Wobbly hedgehog syndrome- a progressive neurodegenerative disease

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

Wobbly hedgehog syndrome- a progressive neurodegenerative disease

Grayson A Doss et al. Exp Neurol. 2023 Oct.

. 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.

PubMed Disclaimer

Conflict of interest statement

Declaration of Competing Interest JS is a co-inventor in the patent # US 9,248,128.

Update of

Wobbly hedgehog syndrome- a progressive neurodegenerative disease.
Doss GA, Radecki DZ, Kethireddy A, Reilly MJ, Pohly AE, August BK, Duncan ID, Samanta J. Doss GA, et al. bioRxiv [Preprint]. 2023 Jul 15:2023.07.13.547983. doi: 10.1101/2023.07.13.547983. bioRxiv. 2023. Update in: Exp Neurol. 2023 Oct;368:114520. doi: 10.1016/j.expneurol.2023.114520. PMID: 37503221 Free PMC article. Updated. Preprint.

Publication types

Actions
Actions

MeSH terms

Actions
Actions
Actions
Actions
Actions
Actions
Actions

Save citation to file

Email citation

Add to Collections

Add to My Bibliography

Your saved search

Create a file for external citation management software

Your RSS Feed

Wobbly hedgehog syndrome- a progressive neurodegenerative disease

Affiliations

Wobbly hedgehog syndrome- a progressive neurodegenerative disease

Authors

Affiliations

Abstract

Conflict of interest statement

Update of

Publication types

MeSH terms

Grants and funding