Results 11 to 20 of about 32,016,499 (171)

Glycosylation, transport, and complex formation of palmitoyl protein thioesterase 1 (PPT1) – distinct characteristics in neurons [PDF]

BMC Cell Biology, 2007
Background Neuronal ceroid lipofuscinoses (NCLs) are collectively the most common type of recessively inherited childhood encephalopathies. The most severe form of NCL, infantile neuronal ceroid lipofuscinosis (INCL), is caused by mutations in the CLN1 ...
Jauhiainen Matti, Saarela Jani, Kopra Outi, Salonen Tarja, von Schantz Carina, Lyly Annina, Kyttälä Aija, Jalanko Anu   +7 more
doaj   +6 more sources

Histochemical localization of palmitoyl protein thioesterase-1 activity. [PDF]

Mol Genet Metab, 2016
Infantile neuronal ceroid lipofuscinosis (INCL, Infantile Batten disease) is an invariably fatal neurodegenerative pediatric disorder caused by an inherited mutation in the PPT1 gene. Patients with INCL lack the lysosomal enzyme palmitoyl protein thioesterase-1 (PPT1, EC 3.1.2.22), resulting in intracellular accumulation of autofluorescent storage ...
Dearborn JT, Ramachandran S, Shyng C, Lu JY, Thornton J, Hofmann SL, Sands MS.   +6 more
europepmc   +5 more sources

Regional and cellular neuropathology in the palmitoyl protein thioesterase-1 null mutant mouse model of infantile neuronal ceroid lipofuscinosis

Neurobiology of Disease, 2004
Infantile neuronal ceroid lipofuscinosis (INCL) is one of a group of fatal hereditary lysosomal storage disorders. Palmitoyl protein thioesterase 1 null mutant mice (PPT1−/−) now exist that accurately recapitulate many important disease features.
Ellen Bible, Praveena Gupta, Sandra L Hofmann, Jonathan D Cooper   +3 more
doaj   +4 more sources

Identification of New Modulators and Inhibitors of Palmitoyl-Protein Thioesterase 1 for CLN1 Batten Disease and Cancer [PDF]

ACS Omega
Palmitoyl-protein thioesterase 1 (PPT1) is an understudied enzyme that is gaining attention due to its role in the depalmitoylation of several proteins involved in neurodegenerative diseases and cancer. PPT1 is overexpressed in several cancers, specifically cholangiocarcinoma and esophageal cancers.
Ana C. Puhl, Renuka Raman, Tammy M. Havener, Eni Minerali, Anthony J. Hickey, Sean Ekins   +5 more
doaj   +4 more sources

Cerebellar pathology and motor deficits in the palmitoyl protein thioesterase 1-deficient mouse. [PDF]

Exp Neurol, 2009
Infantile neuronal ceroid lipofuscinosis (INCL, Infantile Batten Disease) is an inherited, neurodegenerative lysosomal storage disorder. INCL is the result of a CLN1 gene mutation leading to a deficiency in palmitoyl protein thioesterase 1 (PPT1 ...
Macauley SL, Wozniak DF, Kielar C, Tan Y, Cooper JD, Sands MS.   +5 more
europepmc   +6 more sources

Widespread Expression of a Membrane-Tethered Version of the Soluble Lysosomal Enzyme Palmitoyl Protein Thioesterase-1. [PDF]

JIMD Rep, 2017
"Cross-correction," the transfer of soluble lysosomal enzymes between neighboring cells, forms the foundation for therapeutics of lysosomal storage disorders (LSDs). However, "cross-correction" poses a significant barrier to studying the role of specific cell types in LSD pathogenesis.
Shyng C, Macauley SL, Dearborn JT, Sands MS.   +3 more
europepmc   +5 more sources

Palmitoyl-protein Thioesterase 1 in Developmental Plasticity and Infantile Neuronal Ceroid Lipofuscinosis [PDF]

, 2021
Infantile neuronal ceroid lipofuscinosis (CLN1) is a pediatric neurodegenerative disease that affects children and results in death by age 5. Mutations in the neuronal enzyme palmitoyl-protein thioesterase 1 (PPT1) cause CLN1.
Kevin P Koster (11713382)
core   +2 more sources

Palmitoyl protein thioesterase 1 modulates tumor necrosis factor alpha-induced apoptosis. [PDF]

Biochimica et Biophysica Acta (BBA) - Molecular Cell Research, 2009
International audienceInduction of apoptosis by TNF has recently been shown to implicate proteases from lysosomal origin, the cathepsins. Here, we investigated the role in apoptosis of palmitoyl protein thioesterase 1 (PPT1), another lysosomal enzyme ...
Jalanko, Anu, Virginie Garcia, Tardy, Claudine, Nicole Therville, Thierry Levade, Nathalie Andrieu-Abadie, Garcia, Virginie, Anu Jalanko, Therville, Nicole, Andrieu-Abadie, Nathalie, Levade, Thierry, Claudine Tardy, Sabourdy, Frédérique, Frédérique Sabourdy   +13 more
core   +3 more sources

Palmitoyl-Protein Thioesterase 1 (PPT1) Protein, Linked to Neuronal Ceroid Lipofuscinosis 1, Is a Major Constituent of Ageing-Related Human Neuronal Lipofuscin. [PDF]

Neuropathol Appl Neurobiol
ABSTRACT Proteomics of laser‐dissected lipofuscin from aged, healthy brains reveals Palmitoyl‐Protein Thioesterase 1 (PPT1) and other CLN proteins as constituents. PPT1 is increasingly sequestered ...
Anstötz M, Tschirner S, May C, Kösters S, Martin C, Caspers S, Aronica E, Bidmon HJ, Marcus K, Korth C.   +9 more
europepmc   +5 more sources

Status Epilepticus Induces Changes in the Expression and Localization of Endogenous Palmitoyl-Protein Thioesterase 1

Neurobiology of Disease, 2002
Kainic acid (KA)-induced experimental epilepsy, a model of excitotoxicity, leads to selective neuronal death and synaptic restructuring. We used this model to investigate the effects of neuronal hyperactivation on palmitoyl-protein thioesterase 1 (PPT1),
J. Suopanki, M. Lintunen, H. Lahtinen, M. Haltia, P. Panula, M. Baumann, J. Tyynelä   +6 more
doaj   +3 more sources