| review article | Q7318358 |
| scholarly article | Q13442814 |
| P356 | DOI | 10.1002/(SICI)1097-4547(20000415)60:2<133::AID-JNR1>3.0.CO;2-3 |
| P698 | PubMed publication ID | 10740217 |
| P2093 | author name string | S Cho | |
| G Dawson | |||
| P2860 | cites work | Accumulation of dolichol-linked oligosaccharides in ceroid-lipofuscinosis (Batten disease). | Q48151721 |
| Palmitoyl-protein thioesterase gene expression in the developing mouse brain and retina: implications for early loss of vision in infantile neuronal ceroid lipofuscinosis | Q48219067 | ||
| Increases in Bcl-2 and cleavage of caspase-1 and caspase-3 in human brain after head injury. | Q48222559 | ||
| The expression of palmitoyl-protein thioesterase is developmentally regulated in neural tissues but not in nonneural tissues | Q48246454 | ||
| Follow-up study of subunit c of mitochondrial ATP synthase (SCMAS) in Batten disease and in unrelated lysosomal disorders | Q48744657 | ||
| Palmitoylation of endogenous and viral acceptor proteins by fatty acyltransferase (PAT) present in erythrocyte ghosts and in placental membranes | Q50759273 | ||
| A novel assay for lysosomal pepstatin-insensitive proteinase and its application for the diagnosis of late-infantile neuronal ceroid lipofuscinosis. | Q52177657 | ||
| Studies of atypical JNCL suggest overlapping with other NCL forms. | Q52190082 | ||
| BTN1, a Yeast Gene Corresponding to the Human Gene Responsible for Batten's Disease, is Not Essential for Viability, Mitochondrial Function, or Degradation of Mitochondrial ATP Synthase | Q52526313 | ||
| The neuronal ceroid-lipofuscinoses | Q70938428 | ||
| Autoacylation of G protein alpha subunits | Q71514812 | ||
| Specific delay in the degradation of mitochondrial ATP synthase subunit c in late infantile neuronal ceroid lipofuscinosis is derived from cellular proteolytic dysfunction rather than structural alteration of subunit c | Q71658709 | ||
| Sphingolipid activator proteins (SAPs) are stored together with glycosphingolipids in the infantile neuronal ceroid-lipofuscinosis (INCL) | Q72042987 | ||
| Biochemical Characterization of a Palmitoyl Acyltransferase Activity That Palmitoylates Myristoylated Proteins | Q72055049 | ||
| Specific delay of degradation of mitochondrial ATP synthase subunit c in late infantile neuronal ceroid lipofuscinosis (Batten disease) | Q72429926 | ||
| The dystrophic retina in multisystem disorders: the electroretinogram in neuronal ceroid lipofuscinoses | Q77421409 | ||
| Signalling functions of protein palmitoylation | Q77631481 | ||
| Classical late infantile neuronal ceroid lipofuscinosis fibroblasts are deficient in lysosomal tripeptidyl peptidase I | Q22001448 | ||
| Lipid thioesters derived from acylated proteins accumulate in infantile neuronal ceroid lipofuscinosis: correction of the defect in lymphoblasts by recombinant palmitoyl-protein thioesterase | Q24308145 | ||
| Biosynthesis and intracellular targeting of the CLN3 protein defective in Batten disease | Q24310339 | ||
| Association of mutations in a lysosomal protein with classical late-infantile neuronal ceroid lipofuscinosis | Q24311617 | ||
| Human palmitoyl protein thioesterase: evidence for lysosomal targeting of the enzyme and disturbed cellular routing in infantile neuronal ceroid lipofuscinosis | Q24316922 | ||
| A receptor for the selective uptake and degradation of proteins by lysosomes | Q24319797 | ||
| Molecular cloning and expression of palmitoyl-protein thioesterase 2 (PPT2), a homolog of lysosomal palmitoyl-protein thioesterase with a distinct substrate specificity | Q24320100 | ||
| Mutations in the palmitoyl protein thioesterase gene causing infantile neuronal ceroid lipofuscinosis | Q24322669 | ||
| Mutational analysis of the defective protease in classic late-infantile neuronal ceroid lipofuscinosis, a neurodegenerative lysosomal storage disorder | Q24540041 | ||
| Spectrum of mutations in the Batten disease gene, CLN3 | Q24678552 | ||
| The subcellular location of the yeast Saccharomyces cerevisiae homologue of the protein defective in the juvenile form of Batten disease | Q27931549 | ||
| Apoptosis in the pathogenesis and treatment of disease | Q28235731 | ||
| A cytoplasmic acyl-protein thioesterase that removes palmitate from G protein alpha subunits and p21(RAS) | Q28273568 | ||
| Lysosomal targeting of palmitoyl-protein thioesterase | Q28282060 | ||
| Palmitoyl-protein thioesterase, an enzyme implicated in neurodegeneration, is localized in neurons and is developmentally regulated in rat brain | Q28575808 | ||
| A murine model for juvenile NCL: gene targeting of mouse Cln3 | Q28588743 | ||
| 2.2 Mb of contiguous nucleotide sequence from chromosome III of C. elegans | Q29547793 | ||
| Ovine neuronal ceroid lipofuscinosis: a large animal model syntenic with the human neuronal ceroid lipofuscinosis variant CLN6. | Q33681240 | ||
| Coding sequence and exon/intron organization of the canine CLN3 (Batten disease) gene and its exclusion as the locus for ceroid-lipofuscinosis in English setter dogs | Q34747254 | ||
| A yeast model for the study of Batten disease | Q36497992 | ||
| Molecular genetics of palmitoyl-protein thioesterase deficiency in the U.S. | Q37383913 | ||
| Biochemical characterization of a lysosomal protease deficient in classical late infantile neuronal ceroid lipofuscinosis (LINCL) and development of an enzyme-based assay for diagnosis and exclusion of LINCL in human specimens and animal models | Q38467763 | ||
| The dynamic role of palmitoylation in signal transduction | Q40443876 | ||
| Expression of polyglutamine-expanded Huntingtin activates the SEK1-JNK pathway and induces apoptosis in a hippocampal neuronal cell line | Q41000432 | ||
| Specific alterations in levels of mannose 6-phosphorylated glycoproteins in different neuronal ceroid lipofuscinoses | Q41010705 | ||
| Enzymatic and molecular biological analysis of palmitoyl protein thioesterase deficiency in infantile neuronal ceroid lipofuscinosis | Q41029369 | ||
| Batten disease and mitochondrial pathways of proteolysis | Q41115539 | ||
| Low molecular weight storage material in infantile ceroid lipofuscinosis (CLN1). | Q41129060 | ||
| Palmitoyl-protein thioesterase and the molecular pathogenesis of infantile neuronal ceroid lipofuscinosis | Q41129063 | ||
| Reversible palmitoylation of signaling proteins | Q41382874 | ||
| Isolation and chromosomal mapping of a mouse homolog of the Batten disease gene CLN3. | Q42638704 | ||
| P433 | issue | 2 | |
| P407 | language of work or name | English | Q1860 |
| P921 | main subject | protein catabolic process | Q14876077 |
| CLN5 intracellular trafficking protein | Q21109783 | ||
| CLN6 transmembrane ER protein | Q21109834 | ||
| CLN8 transmembrane ER and ERGIC protein | Q21109841 | ||
| Palmitoyl-protein thioesterase 1 | Q21134385 | ||
| tripeptidyl peptidase 1 | Q21136439 | ||
| P304 | page(s) | 133-40 | |
| P577 | publication date | 2000-04-15 | |
| P1433 | published in | Journal of Neuroscience Research | Q6295654 |
| P1476 | title | Batten's disease: clues to neuronal protein catabolism in lysosomes | |
| P478 | volume | 60 |
| Q24290365 | Antisense palmitoyl protein thioesterase 1 (PPT1) treatment inhibits PPT1 activity and increases cell death in LA-N-5 neuroblastoma cells |
| Q29547914 | Autophagy as a regulated pathway of cellular degradation |
| Q28513876 | Biosynthetic processing of cathepsins and lysosomal degradation are abolished in asparaginyl endopeptidase-deficient mice |
| Q48583470 | CLC-3 deficiency leads to phenotypes similar to human neuronal ceroid lipofuscinosis |
| Q45889316 | CNS-directed AAV2-mediated gene therapy ameliorates functional deficits in a murine model of infantile neuronal ceroid lipofuscinosis. |
| Q36510519 | Chaperone-mediated autophagy in aging and disease |
| Q52575698 | Characterisation of lipofuscin-like lysosomal inclusion bodies from human placenta. |
| Q37108109 | Clearing the brain's cobwebs: the role of autophagy in neuroprotection |
| Q45089572 | Electroretinographic and clinicopathologic correlations of retinal dysfunction in infantile neuronal ceroid lipofuscinosis (infantile Batten disease). |
| Q33862796 | Function, therapeutic potential and cell biology of BACE proteases: current status and future prospects |
| Q48397854 | Functional categorization of gene expression changes in the cerebellum of a Cln3-knockout mouse model for Batten disease |
| Q81502786 | Intermediate levels of neuronal palmitoyl-protein Delta-9 desaturase in heterozygotes for murine Batten disease |
| Q43744841 | Involvement of nitric oxide released from microglia-macrophages in pathological changes of cathepsin D-deficient mice. |
| Q34561000 | Looking chloride channels straight in the eye: bestrophins, lipofuscinosis, and retinal degeneration |
| Q30951919 | Metabolic profiles to define the genome: can we hear the phenotypes? |
| Q37126849 | Neuronal pigmented autophagic vacuoles: lipofuscin, neuromelanin, and ceroid as macroautophagic responses during aging and disease |
| Q40309067 | Over-expression of CLN3P, the Batten disease protein, inhibits PANDER-induced apoptosis in neuroblastoma cells: further evidence that CLN3P has anti-apoptotic properties |
| Q44806253 | Profound infantile neuroretinal dysfunction in a heterozygote for the CLN3 genetic defect |
| Q28569453 | The H+-coupled electrogenic lysosomal amino acid transporter LYAAT1 localizes to the axon and plasma membrane of hippocampal neurons |
| Q34129828 | The neuronal ceroid lipofuscinoses: mutations in different proteins result in similar disease |
| Q44186141 | Vitamin E deficiency and metabolic deficits in neuronal ceroid lipofuscinosis described by bioinformatics. |
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