PMID- 30918483
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20201001
IS  - 1662-5102 (Print)
IS  - 1662-5102 (Electronic)
IS  - 1662-5102 (Linking)
VI  - 13
DP  - 2019
TI  - The Interactome of Palmitoyl-Protein Thioesterase 1 (PPT1) Affects Neuronal 
      Morphology and Function.
PG  - 92
LID - 10.3389/fncel.2019.00092 [doi]
LID - 92
AB  - 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.
FAU - Sapir, Tamar
AU  - Sapir T
AD  - Department of Molecular Genetics, Weizmann Institute of Science, Rehovot, Israel.
FAU - Segal, Michal
AU  - Segal M
AD  - Department of Molecular Genetics, Weizmann Institute of Science, Rehovot, Israel.
FAU - Grigoryan, Gayane
AU  - Grigoryan G
AD  - Department of Neurobiology, Weizmann Institute of Science, Rehovot, Israel.
FAU - Hansson, Karin M
AU  - Hansson KM
AD  - Department of Immunotechnology, Lund University, Lund, Sweden.
FAU - James, Peter
AU  - James P
AD  - Department of Immunotechnology, Lund University, Lund, Sweden.
AD  - Turku Centre for Biotechnology (BTK), University of Turku, Turku, Finland.
FAU - Segal, Menahem
AU  - Segal M
AD  - Department of Neurobiology, Weizmann Institute of Science, Rehovot, Israel.
FAU - Reiner, Orly
AU  - Reiner O
AD  - Department of Molecular Genetics, Weizmann Institute of Science, Rehovot, Israel.
LA  - eng
PT  - Journal Article
DEP - 20190313
PL  - Switzerland
TA  - Front Cell Neurosci
JT  - Frontiers in cellular neuroscience
JID - 101477935
PMC - PMC6424868
OTO - NOTNLM
OT  - PPT1
OT  - hippocampal neurons
OT  - mass-spectrometry
OT  - palmitoyl-protein thioesterase 1
OT  - palmitoylation
EDAT- 2019/03/29 06:00
MHDA- 2019/03/29 06:01
PMCR- 2019/01/01
CRDT- 2019/03/29 06:00
PHST- 2018/09/26 00:00 [received]
PHST- 2019/02/25 00:00 [accepted]
PHST- 2019/03/29 06:00 [entrez]
PHST- 2019/03/29 06:00 [pubmed]
PHST- 2019/03/29 06:01 [medline]
PHST- 2019/01/01 00:00 [pmc-release]
AID - 10.3389/fncel.2019.00092 [doi]
PST - epublish
SO  - Front Cell Neurosci. 2019 Mar 13;13:92. doi: 10.3389/fncel.2019.00092. 
      eCollection 2019.