Results 1 to 10 of about 6,658 (119)

Palmitoyl Protein Thioesterase 1 Is Essential for Myogenic Autophagy of C2C12 Skeletal Myoblast [PDF]

Frontiers in Physiology, 2020
Skeletal muscle differentiation is an essential process for the maintenance of muscle development and homeostasis. Reactive oxygen species (ROS) are critical signaling molecules involved in muscle differentiation. Palmitoyl protein thioesterase 1 (PPT1),
Hyeong Rok Yun, Hyeong Rok Yun, Yong Hwa Jo, Yong Hwa Jo, Jieun Kim, Jieun Kim, Ngoc Ngo Yen Nguyen, Ngoc Ngo Yen Nguyen, Yoonhwa Shin, Yoonhwa Shin, Sung Soo Kim, Sung Soo Kim, Tae Gyu Choi, Tae Gyu Choi   +13 more
doaj   +4 more sources

Identifying cellular pathways modulated by Drosophila palmitoyl-protein thioesterase 1 function [PDF]

Neurobiology of Disease, 2010
Infantile-onset Neuronal Ceroid Lipofuscinosis (INCL) is a severe pediatric neurodegenerative disorder produced by mutations in the gene encoding palmitoyl-protein thioesterase 1 (Ppt1).
Stephanie Saja, Haley Buff, Alexis C. Smith, Tiffany S. Williams, Christopher A. Korey   +4 more
doaj   +4 more sources

Depalmitoylation by Palmitoyl-Protein Thioesterase 1 in Neuronal Health and Degeneration [PDF]

Frontiers in Synaptic Neuroscience, 2019
Protein palmitoylation is the post-translational, reversible addition of a 16-carbon fatty acid, palmitate, to proteins. Protein palmitoylation has recently garnered much attention, as it robustly modifies the localization and function of canonical ...
Kevin P. Koster, Akira Yoshii, Akira Yoshii, Akira Yoshii   +3 more
doaj   +2 more sources

Identification of substrates of palmitoyl protein thioesterase 1 highlights roles of depalmitoylation in disulfide bond formation and synaptic function. [PDF]

PLoS Biology, 2022
Loss-of-function mutations in the depalmitoylating enzyme palmitoyl protein thioesterase 1 (PPT1) cause neuronal ceroid lipofuscinosis (NCL), a devastating neurodegenerative disease.
Erica L Gorenberg, Sofia Massaro Tieze, Betül Yücel, Helen R Zhao, Vicky Chou, Gregory S Wirak, Susumu Tomita, TuKiet T Lam, Sreeganga S Chandra   +8 more
doaj   +2 more sources

The Interactome of Palmitoyl-Protein Thioesterase 1 (PPT1) Affects Neuronal Morphology and Function [PDF]

Frontiers in Cellular Neuroscience, 2019
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.
Tamar Sapir, Michal Segal, Gayane Grigoryan, Karin M. Hansson, Peter James, Peter James, Menahem Segal, Orly Reiner   +7 more
doaj   +2 more sources

Reversible Cysteine Acylation Regulates the Activity of Human Palmitoyl-Protein Thioesterase 1 (PPT1). [PDF]

PLoS ONE, 2016
Mutations in the depalmitoylating enzyme gene, PPT1, cause the infantile form of Neuronal Ceroid Lipofuscinosis (NCL), an early onset neurodegenerative disease.
Michal Segal-Salto, Tamar Sapir, Orly Reiner   +2 more
doaj   +2 more sources

Mice deficient in the lysosomal enzyme palmitoyl-protein thioesterase 1 (PPT1) display a complex retinal phenotype [PDF]

Scientific Reports, 2019
Neuronal ceroid lipofuscinosis (NCL) type 1 (CLN1) is a neurodegenerative storage disorder caused by mutations in the gene encoding the lysosomal enzyme palmitoyl-protein thioesterase 1 (PPT1).
Yevgeniya Atiskova, Susanne Bartsch, Tatyana Danyukova, Elke Becker, Christian Hagel, Stephan Storch, Udo Bartsch   +6 more
doaj   +2 more sources

The Batten disease Palmitoyl Protein Thioesterase 1 gene regulates neural specification and axon connectivity during Drosophila embryonic development. [PDF]

PLoS ONE, 2010
Palmitoyl Protein Thioesterase 1 (PPT1) is an essential lysosomal protein in the mammalian nervous system whereby defects result in a fatal pediatric disease called Infantile Neuronal Ceroids Lipofuscinosis (INCL).
Quynh Chu-LaGraff, Cassandra Blanchette, Patrick O'Hern, Cassandra Denefrio   +3 more
doaj   +2 more sources

Dual Regulation of Sprouty 4 Palmitoylation by ZDHHC7 and Palmitoyl-Protein Thioesterase 1: A Potential Therapeutic Strategy for Cisplatin-Resistant Osteosarcoma [PDF]

Research
Background: Osteosarcoma (OS) is a primary malignant bone tumor predominantly affecting adolescents. Chemotherapeutic agents, such as cisplatin, are commonly used in OS treatment; however, drug resistance markedly undermines treatment efficacy and ...
Tianlong Huang, Yifan Chen, Qiangqiang Zhao, Xin Wu, Hongxing Li, Xin Luo, Yang Su, Shengqun Zhang, Pan Liu, Ning Tang   +9 more
doaj   +2 more sources

In a model of Batten disease, palmitoyl protein thioesterase-1 deficiency is associated with brown adipose tissue and thermoregulation abnormalities. [PDF]

PLoS ONE, 2012
Infantile neuronal ceroid lipofuscinosis (INCL) is a fatal neurodegenerative disorder caused by a deficiency of palmitoyl-protein thioesterase-1 (PPT1). We have previously shown that children with INCL have increased risk of hypothermia during anesthesia
Alfia Khaibullina, Nicholas Kenyon, Virginia Guptill, Martha M Quezado, Li Wang, Deloris Koziol, Robert Wesley, Pablo R Moya, Zhongjian Zhang, Arjun Saha, Anil B Mukherjee, Zenaide M N Quezado   +11 more
doaj   +2 more sources