Palmitoyl-protein thioesterase, an enzyme implicated in neurodegeneration, is localized in neurons and is developmentally regulated in rat brain

Palmitoyl-protein thioesterase (PPT) is an enzyme involved in cleavage of palmitate residues from acylated proteins. Mutations in PPT gene cause a severe neurodegenerative disorder, infantile neuronal ceroid-lipofuscinosis (INCL), characterized by loss of cortical neurons. In order to clarify the ro...

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Veröffentlicht in:Neuroscience letters 1999-04, Vol.265 (1), p.53-56
Hauptverfasser: Suopanki, Jaana, Tyynelä, Jaana, Baumann, Marc, Haltia, Matti
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container_title Neuroscience letters
container_volume 265
creator Suopanki, Jaana
Tyynelä, Jaana
Baumann, Marc
Haltia, Matti
description Palmitoyl-protein thioesterase (PPT) is an enzyme involved in cleavage of palmitate residues from acylated proteins. Mutations in PPT gene cause a severe neurodegenerative disorder, infantile neuronal ceroid-lipofuscinosis (INCL), characterized by loss of cortical neurons. In order to clarify the role of PPT and palmitoylation/depalmitoylation in the development of CNS and pathogenesis of infantile neuronal ceroid-lipofuscinosis (INCL), we studied the localization and expression of PPT in developing rats. Using immunohistochemical methods, we show for the first time that PPT is truly localized in neurons. Further, using RT-PCR and Western blotting, we show that expression of PPT in rat brain is developmentally regulated, with increasing expression during the maturation of CNS, reaching the maximum in young adulthood. The presented data support the view of PPT being essential for both development and maintenance of cortical neurons.
doi_str_mv 10.1016/S0304-3940(99)00207-4
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Mutations in PPT gene cause a severe neurodegenerative disorder, infantile neuronal ceroid-lipofuscinosis (INCL), characterized by loss of cortical neurons. In order to clarify the role of PPT and palmitoylation/depalmitoylation in the development of CNS and pathogenesis of infantile neuronal ceroid-lipofuscinosis (INCL), we studied the localization and expression of PPT in developing rats. Using immunohistochemical methods, we show for the first time that PPT is truly localized in neurons. Further, using RT-PCR and Western blotting, we show that expression of PPT in rat brain is developmentally regulated, with increasing expression during the maturation of CNS, reaching the maximum in young adulthood. 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Mutations in PPT gene cause a severe neurodegenerative disorder, infantile neuronal ceroid-lipofuscinosis (INCL), characterized by loss of cortical neurons. In order to clarify the role of PPT and palmitoylation/depalmitoylation in the development of CNS and pathogenesis of infantile neuronal ceroid-lipofuscinosis (INCL), we studied the localization and expression of PPT in developing rats. Using immunohistochemical methods, we show for the first time that PPT is truly localized in neurons. Further, using RT-PCR and Western blotting, we show that expression of PPT in rat brain is developmentally regulated, with increasing expression during the maturation of CNS, reaching the maximum in young adulthood. 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source MEDLINE; ScienceDirect Journals (5 years ago - present)
subjects Animals
Biological and medical sciences
Blotting, Western
Brain development
Development. Senescence. Regeneration. Transplantation
Fundamental and applied biological sciences. Psychology
Gene Expression Regulation, Developmental - physiology
Immunohistochemistry
Infantile neuronal ceroid-lipofuscinosis
Nerve Degeneration
Neurons - chemistry
Palmitoyl-protein thioesterase
Rats
Reverse Transcriptase Polymerase Chain Reaction
RT-PCR, protein expression
Thiolester Hydrolases - analysis
Thiolester Hydrolases - genetics
Vertebrates: nervous system and sense organs
title Palmitoyl-protein thioesterase, an enzyme implicated in neurodegeneration, is localized in neurons and is developmentally regulated in rat brain
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