Axon demyelination and degeneration in a zebrafish spastizin model of hereditary spastic paraplegia

Garg V, André S, Heyer L, Kracht G, Ruhwedel T, Scholz P, Ischebeck T, Werner HB, Dullin C, Engelmann J, Möbius W, et al. (2024)
Open Biology 14(11): 240100.

Zeitschriftenaufsatz | Veröffentlicht | Englisch
 
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Autor*in
Garg, Vranda; André, Selina; Heyer, Luisa; Kracht, Gudrun; Ruhwedel, Torben; Scholz, Patricia; Ischebeck, Till; Werner, Hauke B.; Dullin, Christian; Engelmann, JacobUniBi ; Möbius, Wiebke; Göpfert, Martin C.
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Abstract / Bemerkung
Hereditary spastic paraplegias (HSPs) are a diverse set of neurological disorders characterized by progressive spasticity and weakness in the lower limbs caused by damage to the axons of the corticospinal tract. More than 88 genetic mutations have been associated with HSP, yet the mechanisms underlying these disorders are not well understood. We replicated the pathophysiology of one form of HSP known as spastic paraplegia 15 (SPG15) in zebrafish. This disorder is caused in humans by mutations in the ZFYVE26 gene, which codes for a protein called SPASTIZIN. We show that, in zebrafish, the significant reduction of Spastizin caused degeneration of large motor neurons. Motor neuron degeneration is associated with axon demyelination in the spinal cord and impaired locomotion in the spastizin mutants. Our findings reveal that the reduction in Spastizin compromises axonal integrity and affects the myelin sheath, ultimately recapitulating the pathophysiology of HSPs.
Erscheinungsjahr
2024
Zeitschriftentitel
Open Biology
Band
14
Ausgabe
11
Art.-Nr.
240100
eISSN
2046-2441
Page URI
https://pub.uni-bielefeld.de/record/2994430

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Garg V, André S, Heyer L, et al. Axon demyelination and degeneration in a zebrafish spastizin model of hereditary spastic paraplegia. Open Biology . 2024;14(11): 240100.
Garg, V., André, S., Heyer, L., Kracht, G., Ruhwedel, T., Scholz, P., Ischebeck, T., et al. (2024). Axon demyelination and degeneration in a zebrafish spastizin model of hereditary spastic paraplegia. Open Biology , 14(11), 240100. https://doi.org/10.1098/rsob.240100
Garg, Vranda, André, Selina, Heyer, Luisa, Kracht, Gudrun, Ruhwedel, Torben, Scholz, Patricia, Ischebeck, Till, et al. 2024. “Axon demyelination and degeneration in a zebrafish spastizin model of hereditary spastic paraplegia”. Open Biology 14 (11): 240100.
Garg, V., André, S., Heyer, L., Kracht, G., Ruhwedel, T., Scholz, P., Ischebeck, T., Werner, H. B., Dullin, C., Engelmann, J., et al. (2024). Axon demyelination and degeneration in a zebrafish spastizin model of hereditary spastic paraplegia. Open Biology 14:240100.
Garg, V., et al., 2024. Axon demyelination and degeneration in a zebrafish spastizin model of hereditary spastic paraplegia. Open Biology , 14(11): 240100.
V. Garg, et al., “Axon demyelination and degeneration in a zebrafish spastizin model of hereditary spastic paraplegia”, Open Biology , vol. 14, 2024, : 240100.
Garg, V., André, S., Heyer, L., Kracht, G., Ruhwedel, T., Scholz, P., Ischebeck, T., Werner, H.B., Dullin, C., Engelmann, J., Möbius, W., Göpfert, M.C., Dosch, R., Geurten, B.R.H.: Axon demyelination and degeneration in a zebrafish spastizin model of hereditary spastic paraplegia. Open Biology . 14, : 240100 (2024).
Garg, Vranda, André, Selina, Heyer, Luisa, Kracht, Gudrun, Ruhwedel, Torben, Scholz, Patricia, Ischebeck, Till, Werner, Hauke B., Dullin, Christian, Engelmann, Jacob, Möbius, Wiebke, Göpfert, Martin C., Dosch, Roland, and Geurten, Bart R. H. “Axon demyelination and degeneration in a zebrafish spastizin model of hereditary spastic paraplegia”. Open Biology 14.11 (2024): 240100.

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