Results 81 to 90 of about 32,016,499 (171)

Table_1_The Interactome of Palmitoyl-Protein Thioesterase 1 (PPT1) Affects Neuronal Morphology and Function.docx

, 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.
Orly Reiner (20376), Peter James (348853), Karin M. Hansson (6451355), Tamar Sapir (20381), Michal Segal (6451349), Menahem Segal (342544), Gayane Grigoryan (6451352)   +6 more
core   +1 more source

PPT1 inhibition enhances the antitumor activity of anti–PD-1 antibody in melanoma

JCI Insight, 2020
New strategies are needed to enhance the efficacy of anti–programmed cell death protein antibody (anti–PD-1 Ab) in cancer. Here, we report that inhibiting palmitoyl-protein thioesterase 1 (PPT1), a target of chloroquine derivatives like ...
Gaurav Sharma, Rani Ojha, Estela Noguera-Ortega, Vito W. Rebecca, John Attanasio, Shujing Liu, Shengfu Piao, Jennifer J. Lee, Michael C. Nicastri, Sandra L. Harper, Amruta Ronghe, Vaibhav Jain, Jeffrey D. Winkler, David W. Speicher, Jerome Mastio, Phyllis A. Gimotty, Xiaowei Xu, E. John Wherry, Dmitry I. Gabrilovich, Ravi K. Amaravadi   +19 more
doaj   +1 more source

Energetic stress in combination with impaired fatty acid oxidation induces sequestration of CoA and adaptation of CoA metabolism

The FEBS Journal, Volume 293, Issue 12, Page 3565-3587, June 2026.
Computational modelling and in vitro liver cell experiments indicate that medium‐chain acyl‐CoA dehydrogenase (MCAD) deficiency causes an accumulation of (especially medium‐chain) acyl‐CoAs at the cost of free CoA (CoASH). A substantial decrease in CoASH impairs flux through many pathways essential for energy homeostasis.
Ligia Akemi Kiyuna, Christoff Odendaal, Madhulika Singh, Albert Gerding, Miriam Langelaar‐Makkinje, Marianne van der Zwaag, Asmara Drachman, Vladimíra Cetkovská, Gaby Liem Foeng Kioen, Anne‐Claire M. F. Martines, Nicolette C. A. Huijkman, Hein Schepers, Bart van de Sluis, Dirk‐Jan Reijngoud, Ody C. M. Sibon, Amy C. Harms, Thomas Hankemeier, Barbara M. Bakker   +17 more
wiley   +1 more source

Palmitoyl protein thioesterase-1 deficiency impairs synaptic vesicle recycling at nerve terminals, contributing to neuropathology in humans and mice [PDF]

Journal of Clinical Investigation, 2008
Neuronal ceroid lipofuscinoses represent the most common childhood neurodegenerative storage disorders. Infantile neuronal ceroid lipofuscinosis (INCL) is caused by palmitoyl protein thioesterase-1 (PPT1) deficiency. Although INCL patients show signs of abnormal neurotransmission, manifested by myoclonus and seizures, the molecular mechanisms by which ...
Sung-Jo, Kim, Zhongjian, Zhang, Chinmoy, Sarkar, Pei-Chih, Tsai, Yi-Ching, Lee, Louis, Dye, Anil B, Mukherjee   +6 more
openaire   +2 more sources

Chemical Proteomic Analysis of Serine Hydrolase Activity in Niemann-Pick Type C Mouse Brain

Frontiers in Neuroscience, 2018
The endocannabinoid system (ECS) is considered to be an endogenous protective system in various neurodegenerative diseases. Niemann-Pick type C (NPC) is a neurodegenerative disease in which the role of the ECS has not been studied yet.
Eva J. van Rooden, Annelot C. M. van Esbroeck, Marc P. Baggelaar, Hui Deng, Bogdan I. Florea, André R. A. Marques, Roelof Ottenhoff, Rolf G. Boot, Herman S. Overkleeft, Johannes M. F. G. Aerts, Mario van der Stelt   +10 more
doaj   +1 more source

Human recombinant palmitoyl-protein thioesterase-1 (PPT1) for preclinical evaluation of enzyme replacement therapy for infantile neuronal ceroid lipofuscinosis. [PDF]

Mol Genet Metab, 2010
Infantile neuronal ceroid lipofuscinosis (INCL, also known as Haltia-Santavuori disease) is a lysosomal storage disorder of infants and children characterized by blindness, seizures and a progressive neurodegenerative course. Recent clinical trials have involved neural stem cells and gene therapy directed to the central nervous system; however, enzyme ...
Lu JY, Hu J, Hofmann SL.
europepmc   +4 more sources

Quantitative Analysis Reveals Hitchhiking Drives Polysorbate Hydrolase Persistence Via Host Cell Protein–Antibody Interactions

Biotechnology and Bioengineering, Volume 123, Issue 5, Page 1224-1235, May 2026.
ABSTRACT Polysorbate‐degrading host cell proteins (HCPs) represent a critical challenge in the manufacturing of monoclonal antibody therapeutics due to their potential to persist during downstream processing. While their enzymatic activity has been characterized, the role of direct HCP‐mAb interactions, particularly those involving polysorbate ...
Melanie Maier, Lukas Griesinger, Matthias Franzreb, Simon Kluters   +3 more
wiley   +1 more source

Lysosomal lipid peroxidation regulates tumor immunity

The Journal of Clinical Investigation, 2023
Lysosomal inhibition elicited by palmitoyl-protein thioesterase 1 (PPT1) inhibitors such as DC661 can produce cell death, but the mechanism for this is not completely understood.
Monika Bhardwaj, Jennifer J. Lee, Amanda M. Versace, Sandra L. Harper, Aaron R. Goldman, Mary Ann S. Crissey, Vaibhav Jain, Mahendra Pal Singh, Megane Vernon, Andrew E. Aplin, Seokwoo Lee, Masao Morita, Jeffrey D. Winkler, Qin Liu, David W. Speicher, Ravi K. Amaravadi   +15 more
doaj   +1 more source

Palmitoylation‐Mediated Ubiquitination of SRPK1 Regulates Ferroptosis in High‐Fat‐Induced Erectile Dysfunction

Advanced Science, Volume 13, Issue 19, 2 April 2026.
Elevated exogenous palmitic acid promotes the S‐palmitoylation of SRPK1 in endothelial cells, a dynamic process governed by ZDHHC24 and APT1. This post‐translational modification strengthens the interaction between SRPK1 and the E3 ubiquitin ligase MIB1, thereby facilitating the proteasomal degradation of SRPK1.
Xiao‐Hui Tan, Ke‐Fan Li, Yi‐Ming Yuan, Man‐Cheng Xia, Fang‐Zhou Zhao, Hong‐Gang Ying, Zhuo Zhou, Peng‐Chao Gao, Guo‐Qing Xie, Xue‐Song Li, Hui Jiang, Rui‐Li Guan   +11 more
wiley   +1 more source

Progressive retinal degeneration and glial activation in the Cln6nclf mouse model of neuronal ceroid lipofuscinosis : a beneficial effect of DHA and Curcumin supplementation [PDF]

, 2013
Neuronal ceroid lipofuscinosis (NCL) is a group of neurodegenerative lysosomal storage disorders characterized by vision loss, mental and motor deficits, and spontaneous seizures.
Monica Langiu, Myriam Mirza, Langiu, Monica, Tamm, Ernst R., Mirza, Myriam, Langmann, Thomas, Ernst R. Tamm, Aleksandra Somogyi, Marcus Karlstetter, Mika O. Ruonala, Volz, Cornelia, Jägle, Herbert, Herbert Jägle, Ernst R Tamm, Somogyi, Aleksandra, Cornelia Volz, Thomas Langmann, Mika O Ruonala, Karlstetter, Marcus, Ruonala, Mika O.   +19 more
core   +2 more sources