Lipid thioesters derived from acylated proteins accumulate in infantile neuronal ceroid lipofuscinosis: correction of the defect in lymphoblasts by recombinant palmitoyl-protein thioesterase
about
Characterization of a human MHC class III region gene product with S-thioesterase activityCloning, expression, and cellular localization of a human prenylcysteine lyaseBatten's disease: clues to neuronal protein catabolism in lysosomesIsolation and characterization of a prenylcysteine lyase from bovine brainBiosynthesis and intracellular targeting of the CLN3 protein defective in Batten diseaseMolecular cloning and expression of palmitoyl-protein thioesterase 2 (PPT2), a homolog of lysosomal palmitoyl-protein thioesterase with a distinct substrate specificityDisruption of PPT1 or PPT2 causes neuronal ceroid lipofuscinosis in knockout miceThe crystal structure of palmitoyl protein thioesterase 1 and the molecular basis of infantile neuronal ceroid lipofuscinosisStructural basis for the insensitivity of a serine enzyme (palmitoyl-protein thioesterase) to phenylmethylsulfonyl fluorideCharacterization of Saccharomyces cerevisiae acyl-protein thioesterase 1, the enzyme responsible for G protein alpha subunit deacylation in vivo.Prenylcysteine lyase deficiency in mice results in the accumulation of farnesylcysteine and geranylgeranylcysteine in brain and liverComprehensive functional characterization of murine infantile Batten disease including Parkinson-like behavior and dopaminergic markers.Neuroprotection and lifespan extension in Ppt1(-/-) mice by NtBuHA: therapeutic implications for INCLGene expression profiling in a mouse model of infantile neuronal ceroid lipofuscinosis reveals upregulation of immediate early genes and mediators of the inflammatory responseHuman recombinant palmitoyl-protein thioesterase-1 (PPT1) for preclinical evaluation of enzyme replacement therapy for infantile neuronal ceroid lipofuscinosis.Dynamic palmitoylation and the role of DHHC proteins in T cell activation and anergy.Lysosomal prenylcysteine lyase is a FAD-dependent thioether oxidase.Omega-3 and omega-6 fatty acids suppress ER- and oxidative stress in cultured neurons and neuronal progenitor cells from mice lacking PPT1.Oral cysteamine bitartrate and N-acetylcysteine for patients with infantile neuronal ceroid lipofuscinosis: a pilot studyA mutation in canine PPT1 causes early onset neuronal ceroid lipofuscinosis in a Dachshund.Pathogenesis and therapies for infantile neuronal ceroid lipofuscinosis (infantile CLN1 disease).Palmitoyl-protein thioesterase 1 deficiency in Drosophila melanogaster causes accumulation of abnormal storage material and reduced life spanMice homozygous for c.451C>T mutation in Cln1 gene recapitulate INCL phenotype.Stop codon read-through with PTC124 induces palmitoyl-protein thioesterase-1 activity, reduces thioester load and suppresses apoptosis in cultured cells from INCL patients.Evaluation of neurodegeneration in a mouse model of infantile batten disease by magnetic resonance imaging and magnetic resonance spectroscopy.Cln1 gene disruption in mice reveals a common pathogenic link between two of the most lethal childhood neurodegenerative lysosomal storage disordersIntravenous high-dose enzyme replacement therapy with recombinant palmitoyl-protein thioesterase reduces visceral lysosomal storage and modestly prolongs survival in a preclinical mouse model of infantile neuronal ceroid lipofuscinosis.Palmitoylation-induced aggregation of cysteine-string protein mutants that cause neuronal ceroid lipofuscinosisDisruption of PPT2 in mice causes an unusual lysosomal storage disorder with neurovisceral featuresThematic review series: lipid posttranslational modifications. Lysosomal metabolism of lipid-modified proteins.Fat chance! Getting a grip on a slippery modification.RAGE signaling contributes to neuroinflammation in infantile neuronal ceroid lipofuscinosisProtein acyl thioesterases (Review).Molecular genetics of palmitoyl-protein thioesterase deficiency in the U.S.pdf1, a palmitoyl protein thioesterase 1 Ortholog in Schizosaccharomyces pombe: a yeast model of infantile Batten disease.Analysis of NCL Proteins from an Evolutionary Standpoint.Palmitoylation of the synaptic vesicle fusion machinery.Misrouting of v-ATPase subunit V0a1 dysregulates lysosomal acidification in a neurodegenerative lysosomal storage disease model.Update of the mutation spectrum and clinical correlations of over 360 mutations in eight genes that underlie the neuronal ceroid lipofuscinoses.Synergistic effects of treating the spinal cord and brain in CLN1 disease.
P2860
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P2860
Lipid thioesters derived from acylated proteins accumulate in infantile neuronal ceroid lipofuscinosis: correction of the defect in lymphoblasts by recombinant palmitoyl-protein thioesterase
description
1996 nî lūn-bûn
@nan
1996 թուականի Սեպտեմբերին հրատարակուած գիտական յօդուած
@hyw
1996 թվականի սեպտեմբերին հրատարակված գիտական հոդված
@hy
1996年の論文
@ja
1996年論文
@yue
1996年論文
@zh-hant
1996年論文
@zh-hk
1996年論文
@zh-mo
1996年論文
@zh-tw
1996年论文
@wuu
name
Lipid thioesters derived from ...... palmitoyl-protein thioesterase
@ast
Lipid thioesters derived from ...... palmitoyl-protein thioesterase
@en
Lipid thioesters derived from ...... palmitoyl-protein thioesterase
@en-gb
Lipid thioesters derived from ...... palmitoyl-protein thioesterase
@nl
type
label
Lipid thioesters derived from ...... palmitoyl-protein thioesterase
@ast
Lipid thioesters derived from ...... palmitoyl-protein thioesterase
@en
Lipid thioesters derived from ...... palmitoyl-protein thioesterase
@en-gb
Lipid thioesters derived from ...... palmitoyl-protein thioesterase
@nl
prefLabel
Lipid thioesters derived from ...... palmitoyl-protein thioesterase
@ast
Lipid thioesters derived from ...... palmitoyl-protein thioesterase
@en
Lipid thioesters derived from ...... palmitoyl-protein thioesterase
@en-gb
Lipid thioesters derived from ...... palmitoyl-protein thioesterase
@nl
P2093
P2860
P921
P356
P1476
Lipid thioesters derived from ...... palmitoyl-protein thioesterase
@en
P2093
L A Verkruyse
S L Hofmann
P2860
P304
P356
10.1073/PNAS.93.19.10046
P407
P577
1996-09-17T00:00:00Z