| PPT1 Promotes Growth and Inhibits Ferroptosis of Oral Squamous Cell Carcinoma Cells. | PPT1 Promotes Growth and Inhibits Ferroptosis of Oral Squamous Cell Carcinoma Cells.
Luo Q, Hu S, Tang Y, Yang D, Chen Q. | 09/30/2024 |
| Identification of PPT1 as a lysosomal core gene with prognostic value in hepatocellular carcinoma. | Identification of PPT1 as a lysosomal core gene with prognostic value in hepatocellular carcinoma.
Tian W, Li C, Ren J, Li P, Zhao J, Li S, Dong D., Free PMC Article | 05/26/2023 |
| Computational and structural investigation of Palmitoyl-Protein Thioesterase 1 (PPT1) protein causing Neuronal Ceroid Lipofuscinoses (NCL). | Computational and structural investigation of Palmitoyl-Protein Thioesterase 1 (PPT1) protein causing Neuronal Ceroid Lipofuscinoses (NCL).
Thirumal Kumar D, Shaikh N, Udhaya Kumar S, George Priya Doss C. | 10/1/2022 |
| Human INCL fibroblasts display abnormal mitochondrial and lysosomal networks and heightened susceptibility to ROS-induced cell death. | Human INCL fibroblasts display abnormal mitochondrial and lysosomal networks and heightened susceptibility to ROS-induced cell death.
Balouch B, Nagorsky H, Pham T, LaGraff JT, Chu-LaGraff Q., Free PMC Article | 07/10/2021 |
| High PPT1 expression is associated with cancer. | PPT1 Promotes Tumor Growth and Is the Molecular Target of Chloroquine Derivatives in Cancer.
Rebecca VW, Nicastri MC, Fennelly C, Chude CI, Barber-Rotenberg JS, Ronghe A, McAfee Q, McLaughlin NP, Zhang G, Goldman AR, Ojha R, Piao S, Noguera-Ortega E, Martorella A, Alicea GM, Lee JJ, Schuchter LM, Xu X, Herlyn M, Marmorstein R, Gimotty PA, Speicher DW, Winkler JD, Amaravadi RK., Free PMC Article | 03/28/2020 |
| The results demonstrate that these patient iPSC derived NCL NSCs are valid cell- based disease models with characteristic disease phenotypes that can be used for study of disease pathophysiology and drug development. | Neural stem cells for disease modeling and evaluation of therapeutics for infantile (CLN1/PPT1) and late infantile (CLN2/TPP1) neuronal ceroid lipofuscinoses.
Sima N, Li R, Huang W, Xu M, Beers J, Zou J, Titus S, Ottinger EA, Marugan JJ, Xie X, Zheng W., Free PMC Article | 03/16/2019 |
| the study contributes four novel variants to the spectrum of PPT1 gene mutations and eight novel variants to the TPP1 gene mutation data in neuronal ceroid lipofuscinoses type I and type II | Batten disease: biochemical and molecular characterization revealing novel PPT1 and TPP1 gene mutations in Indian patients.
Sheth J, Mistri M, Bhavsar R, Pancholi D, Kamate M, Gupta N, Kabra M, Mehta S, Nampoothiri S, Thakker A, Jain V, Shah R, Sheth F., Free PMC Article | 01/19/2019 |
| Targeting PPT1 blocks mTOR signaling in a manner distinct from catalytic inhibitors, while concurrently inhibiting autophagy, thereby providing a new strategy for cancer therapy. | A Unified Approach to Targeting the Lysosome's Degradative and Growth Signaling Roles.
Rebecca VW, Nicastri MC, McLaughlin N, Fennelly C, McAfee Q, Ronghe A, Nofal M, Lim CY, Witze E, Chude CI, Zhang G, Alicea GM, Piao S, Murugan S, Ojha R, Levi SM, Wei Z, Barber-Rotenberg JS, Murphy ME, Mills GB, Lu Y, Rabinowitz J, Marmorstein R, Liu Q, Liu S, Xu X, Herlyn M, Zoncu R, Brady DC, Speicher DW, Winkler JD, Amaravadi RK., Free PMC Article | 06/16/2018 |
| the combination of elevated glycolysis and deficient MRPL13 activity was closely linked to CLN1-mediated tumor activity in human hepatoma cells | Lactate-mediated mitoribosomal defects impair mitochondrial oxidative phosphorylation and promote hepatoma cell invasiveness.
Lee YK, Lim JJ, Jeoun UW, Min S, Lee EB, Kwon SM, Lee C, Yoon G., Free PMC Article | 01/6/2018 |
| Proteomics analysis on isolated cilia revealed 660 proteins, which differed in their abundance levels between wild type and Ppt1 knock out. | Proteomics insights into infantile neuronal ceroid lipofuscinosis (CLN1) point to the involvement of cilia pathology in the disease.
Segal-Salto M, Hansson K, Sapir T, Kaplan A, Levy T, Schweizer M, Frotscher M, James P, Reiner O. | 10/21/2017 |
| we reveal the existence of a positive feedback loop, where palmitoylation of PPT1 results in decreased activity and subsequent elevation in the amount of palmitoylated proteins. | Reversible Cysteine Acylation Regulates the Activity of Human Palmitoyl-Protein Thioesterase 1 (PPT1).
Segal-Salto M, Sapir T, Reiner O., Free PMC Article | 07/2/2016 |
| analysis of the palmitoyl protein thioesterase 1 interactome in SH-SY5Y human neuroblastoma cells | Proteomic analysis of the palmitoyl protein thioesterase 1 interactome in SH-SY5Y human neuroblastoma cells.
Scifo E, Szwajda A, Soliymani R, Pezzini F, Bianchi M, Dapkunas A, Dębski J, Uusi-Rauva K, Dadlez M, Gingras AC, Tyynelä J, Simonati A, Jalanko A, Baumann MH, Lalowski M. | 04/16/2016 |
| Data (including data from knockout mice) suggest that deficiency of PPT1 leads to accumulation of granular osmiophilic deposits in many cell types, especially in astrocytes. [review-like article] | Astrocytosis in infantile neuronal ceroid lipofuscinosis: friend or foe?
Shyng C, Sands MS. | 06/6/2015 |
| Data suggest that human monocytes and macrophages express PPT1; PPT1 appears to contribute 32-40% of 2-arachidonylglycerol hydrolysis activity in THP1 monocyte cell line. | Identification of palmitoyl protein thioesterase 1 in human THP1 monocytes and macrophages and characterization of unique biochemical activities for this enzyme.
Wang R, Borazjani A, Matthews AT, Mangum LC, Edelmann MJ, Ross MK., Free PMC Article | 02/15/2014 |
| This neuroimaging finding in PPT1-related neuronal ceroid lipofuscinosis was not previously reported. | Novel neuroimaging finding in palmitoyl protein thioesterase-1-related neuronal ceroid lipofuscinosis.
Kamate M, Hattiholi V. | 09/1/2012 |
| Stop codon read-through with PTC124 induces palmitoyl-protein thioesterase-1 activity, reduces thioester load and suppresses apoptosis in cultured cells from Infantile neuronal ceroid lipofuscinosis patients. | Stop codon read-through with PTC124 induces palmitoyl-protein thioesterase-1 activity, reduces thioester load and suppresses apoptosis in cultured cells from INCL patients.
Sarkar C, Zhang Z, Mukherjee AB., Free PMC Article | 03/3/2012 |
| Results describe the correlation between the three-dimensional structural changes in mutant palmitoyl protein thioesterase 1 and biochemical phenotypes. | Structural basis of neuronal ceroid lipofuscinosis 1.
Ohno K, Saito S, Sugawara K, Suzuki T, Togawa T, Sakuraba H. | 10/23/2010 |
| study presents clinical and diagnostic investigations in six children with variant late infantile neuronal ceroid lipofuscinosis and mutations in CLN1 including a new large-scale deletion on one allele | Variant late infantile neuronal ceroid lipofuscinosis because of CLN1 mutations.
Simonati A, Tessa A, Bernardina BD, Biancheri R, Veneselli E, Tozzi G, Bonsignore M, Grosso S, Piemonte F, Santorelli FM. | 01/21/2010 |
| Using shotgun mass spectrometry, we found this protein differentially expressed in the dorsolateral prefrontal cortex from patients with schizophrenia. | Prefrontal cortex shotgun proteome analysis reveals altered calcium homeostasis and immune system imbalance in schizophrenia.
Martins-de-Souza D, Gattaz WF, Schmitt A, Rewerts C, Maccarrone G, Dias-Neto E, Turck CW. | 01/28/2009 |
| Observational study of gene-disease association. (HuGE Navigator) | See all PubMed (2) articlesMutations in CLN7/MFSD8 are a common cause of variant late-infantile neuronal ceroid lipofuscinosis.
Kousi M, Siintola E, Dvorakova L, Vlaskova H, Turnbull J, Topcu M, Yuksel D, Gokben S, Minassian BA, Elleder M, Mole SE, Lehesjoki AE. Polymorphisms in genes involved in neurodevelopment may be associated with altered brain morphology in schizophrenia: preliminary evidence.
Gregório SP, Sallet PC, Do KA, Lin E, Gattaz WF, Dias-Neto E. | 01/11/2009 |
| Palmitoyl protein thioesterase-1 deficiency impairs synaptic vesicle recycling at nerve terminals in humans and mice | Palmitoyl protein thioesterase-1 deficiency impairs synaptic vesicle recycling at nerve terminals, contributing to neuropathology in humans and mice.
Kim SJ, Zhang Z, Sarkar C, Tsai PC, Lee YC, Dye L, Mukherjee AB., Free PMC Article | 01/21/2010 |
| there is a close correlation between CLN2 and CLN1 expression and colorectal carcinoma progression and metastasis and suggest that they may be potential molecular targets | Overexpression in colorectal carcinoma of two lysosomal enzymes, CLN2 and CLN1, involved in neuronal ceroid lipofuscinosis.
Tsukamoto T, Iida J, Dobashi Y, Furukawa T, Konishi F. | 01/21/2010 |
| ER stress due to PPT1-deficiency increases ROS and disrupts calcium homeostasis activating caspase-9 and caspase-9 activation mediates caspase-3 activation and apoptosis contributing to rapid neurodegeneration in INCL. | Palmitoyl-protein thioesterase-1 deficiency leads to the activation of caspase-9 and contributes to rapid neurodegeneration in INCL.
Kim SJ, Zhang Z, Lee YC, Mukherjee AB. | 01/21/2010 |
| The crystal structure of palmitoyl protein thioesterase-2 (PPT2) reveals the basis for divergent substrate specificities of the two lysosomal thioesterases, PPT1 and PPT2. | The crystal structure of palmitoyl protein thioesterase-2 (PPT2) reveals the basis for divergent substrate specificities of the two lysosomal thioesterases, PPT1 and PPT2.
Calero G, Gupta P, Nonato MC, Tandel S, Biehl ER, Hofmann SL, Clardy J. | 01/21/2010 |
| The clinical, biochemical, and molecular genetic aspects of lysosomal storage disorders are discussed in this review | Neuronal ceroid lipofuscinoses caused by defects in soluble lysosomal enzymes (CLN1 and CLN2).
Hofmann SL, Atashband A, Cho SK, Das AK, Gupta P, Lu JY. | 01/21/2010 |