Results 31 to 40 of about 32,016,499 (171)

Mutations in the palmitoyl-protein thioesterase gene (PPT; CLN1) causing juvenile neuronal ceroid lipofuscinosis with granular osmiophilic deposits.

, 1998
A subtype of neuronal ceroid lipofuscinosis (NCL) is well recognized which has a clinical course consistent with juvenile NCL (JNCL) but the ultrastructural characteristics of infantile NCL (INCL): granular osmiophilic deposits (GROD).
Mitchison, H, Martin, J, Munroe, P, Becerra, C, Lake, B, Mole, S, Williams, R, Philippart, M, O'Rawe, A, Hofmann, S, Gardiner, R, Andermann, F, Stephenson, J, Andermann, E, Crow, YJ, Hofman, I, Taschner, P   +16 more
core   +12 more sources

PPT1 Reduction Contributes to Erianin-Induced Growth Inhibition in Oral Squamous Carcinoma Cells

Frontiers in Cell and Developmental Biology, 2021
The anticancer properties of erianin have been recently discovered. However, the antitumor effect of erianin in oral squamous cell carcinoma (OSCC) remains unclear.
Qingqiong Luo, Xiaoyan Li, Guifang Gan, Meng Yang, Xu Chen, Fuxiang Chen   +5 more
doaj   +1 more source

Computational exploration of palmitoyl-protein thioesterase 1 inhibition by Juniperus phoenicea L. for anti-dementia treatment. [PDF]

J Taibah Univ Med Sci
Dementia, a growing concern globally, affects more than 55 million people-a number projected to rise to 152 million by 2050. Current medications target Alzheimer's disease, the most prevalent form of dementia. This study investigated Juniperus phoenicea L., a plant used in traditional Chinese medicine, as a potential inhibitor of palmitoyl-protein ...
Irsal RAP, Gholam GM, Dwicesaria MA, Mansyah TF, Chairunisa F.   +4 more
europepmc   +3 more sources

Identification of palmitoyl protein thioesterase 1 in human THP1 monocytes and macrophages and characterization of unique biochemical activities for this enzyme. [PDF]

Biochemistry, 2013
The profiles of serine hydrolases in human and mouse macrophages are similar yet different. For instance, human macrophages express high levels of carboxylesterase 1 (CES1), whereas mouse macrophages have minimal amounts of the orthologous murine CES1.
Wang R, Borazjani A, Matthews AT, Mangum LC, Edelmann MJ, Ross MK.   +5 more
europepmc   +4 more sources

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

, 2022
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.
Hansson KM, Reiner O, James P, Sapir T, Segal M, Grigoryan G   +5 more
core   +1 more source

Evaluating palmitoyl-protein thioesterase 1 in oral squamous cell carcinoma: A novel indicator of tumor behavior and therapeutic response. [PDF]

World J Exp Med
BACKGROUND Oral squamous cell carcinoma (OSCC) poses a major health burden, with frequent late-stage diagnoses and limited prognostic tools. Palmitoyl-protein thioesterase (PPT)1, a lysosomal enzyme, has been implicated in tumor biology.
Kumar KCP, Sahoo S, Adhya AK, Gaikwad MR, Ganapathy A, Ravi PK.   +5 more
europepmc   +3 more sources

Dimeric quinacrines as chemical tools to identify PPT1, a new regulator of autophagy in cancer cells

Molecular & Cellular Oncology, 2018
DQ661 is a novel dimeric quinacrine that affects multiple lysosomal functions (autophagy and macropinocytosis) and mTORC1 (mechanistic target of rapamycin) activity by specifically targeting protein-palmitoyl thioesterase 1 (PPT1).
Michael C. Nicastri, Vito W. Rebecca, Ravi K. Amaravadi, Jeffrey D. Winkler   +3 more
doaj   +1 more source

Palmitoyl-protein thioesterase deficiency in fibroblasts of individuals with infantile neuronal ceroid lipofuscinosis and I-cell disease [PDF]

, 1997
Mutations in the gene encoding a recently described lysosomal enzyme, palmitoyl-protein thioesterase (PPT), have recently been shown to result in the neurodegenerative disorder, infantile neuronal ceroid lipofuscinosis (INCL).
Hofmann, Sandra L, Marvin R Natowicz, Verkruyse, Linda A, Natowicz, Marvin R, Linda A Verkruyse, Sandra L Hofmann   +5 more
core   +1 more source

The crystal structure of palmitoyl protein thioesterase 1 and the molecular basis of infantile neuronal ceroid lipofuscinosis [PDF]

Proceedings of the National Academy of Sciences, 2000
Mutations in palmitoyl-protein thioesterase 1 (PPT1), a lysosomal enzyme that removes fatty acyl groups from cysteine residues in modified proteins, cause the fatal inherited neurodegenerative disorder infantile neuronal ceroid lipofuscinosis. The accumulation of undigested substrates leads to the formation of neuronal storage bodies that are ...
J J, Bellizzi, J, Widom, C, Kemp, J Y, Lu, A K, Das, S L, Hofmann, J, Clardy   +6 more
openaire   +2 more sources

Palmitoylation-induced aggregation of cysteine-string protein mutants that cause neuronal ceroid lipofuscinosis [PDF]

, 2012
Recently, mutations in the DNAJC5 gene encoding cysteine-string protein alpha (CSPα) were identified to cause the neurodegenerative disorder adult-onset neuronal ceroid lipofuscinosis.
Greaves, J., Chamberlain, Luke, Chamberlain, L. H., Grefen, C., Lemonidis, K., Cruchaga, Carlos, Greaves, Jennifer, Grefen, Christopher, Lemonidis, Kimon, Gorleku, Oforiwa, Cruchaga, C., Gorleku, O. A.   +11 more
core   +1 more source