The interactome of palmitoyl-protein thioesterase 1 (PPT1) affects neuronal morphology and function

Tamar Sapir, Michal Segal, Gayane Grigoryan, Karin M. Hansson, Peter James, Menahem Segal, Orly Reiner

Forskningsoutput: TidskriftsbidragArtikel i vetenskaplig tidskriftPeer review

16 Citeringar (SciVal)

Sammanfattning

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.

Originalspråkengelska
Artikelnummer92
TidskriftFrontiers in Cellular Neuroscience
Volym13
DOI
StatusPublished - 2019 jan. 29

Ämnesklassifikation (UKÄ)

  • Neurovetenskaper

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