A1 Refereed original research article in a scientific journal
The Interactome of Palmitoyl-Protein Thioesterase 1 (PPT1) Affects Neuronal Morphology and Function
Authors: Sapir T, Segal M, Grigoryan G, Hansson KM, James P, Segal M, Reiner O
Publisher: FRONTIERS MEDIA SA
Publication year: 2019
Journal: Frontiers in Cellular Neuroscience
Journal name in source: FRONTIERS IN CELLULAR NEUROSCIENCE
Journal acronym: FRONT CELL NEUROSCI
Article number: ARTN 92
Volume: 13
Number of pages: 14
ISSN: 1662-5102
eISSN: 1662-5102
DOI: https://doi.org/10.3389/fncel.2019.00092
Web address : https://www.frontiersin.org/articles/10.3389/fncel.2019.00092/full
Self-archived copy’s web address: https://research.utu.fi/converis/portal/detail/Publication/39958058
Palmitoyl-protein thioesterase 1 (PPT1) is a depalmitoylation enzyme that is mutated in cases of neuronal ceroid lipofuscinosis (NCL). The hallmarks of the disease include progressive neurodegeneration and blindness, as well as seizures. In the current study, we identified 62 high-confident PPT1-binding proteins. These proteins included a self-interaction of PPT1, two V-type ATPases, calcium voltage-gated channels, cytoskeletal proteins and others. Pathway analysis suggested their involvement in seizures and neuronal morphology. We then proceeded to demonstrate that hippocampal neurons from Ppt1-/- mice exhibit structural deficits, and further investigated electrophysiology parameters in the hippocampi of mutant mice, both in brain slices and dissociated postnatal primary cultures. Our studies reveal new mechanistic features involved in the pathophysiology of this devastating neurodegenerative disease.
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