Classical late infantile neuronal ceroid lipofuscinosis fibroblasts are deficient in lysosomal tripeptidyl peptidase I
about
Purification and characterization of bovine brain lysosomal pepstatin-insensitive proteinase, the gene product deficient in the human late-infantile neuronal ceroid lipofuscinosisBatten's disease: clues to neuronal protein catabolism in lysosomesThe human CLN2 protein/tripeptidyl-peptidase I is a serine protease that autoactivates at acidic pHElevated lysosomal pH in neuronal ceroid lipofuscinoses (NCLs)The gene mutated in variant late-infantile neuronal ceroid lipofuscinosis (CLN6) and in nclf mutant mice encodes a novel predicted transmembrane proteinDefective endoplasmic reticulum-resident membrane protein CLN6 affects lysosomal degradation of endocytosed arylsulfatase AMutational analysis of the defective protease in classic late-infantile neuronal ceroid lipofuscinosis, a neurodegenerative lysosomal storage disorderMoving towards effective therapeutic strategies for Neuronal Ceroid LipofuscinosisThe crystal structure of palmitoyl protein thioesterase 1 and the molecular basis of infantile neuronal ceroid lipofuscinosisCrystal Structure and Autoactivation Pathway of the Precursor Form of Human Tripeptidyl-peptidase 1, the Enzyme Deficient in Late Infantile Ceroid LipofuscinosisTwo novel CLN2 gene mutations in a Chinese patient with classical late-infantile neuronal ceroid lipofuscinosisProduction and characterization of recombinant human CLN2 protein for enzyme-replacement therapy in late infantile neuronal ceroid lipofuscinosisA critical tryptophan and Ca2+ in activation and catalysis of TPPI, the enzyme deficient in classic late-infantile neuronal ceroid lipofuscinosisLysosomal degradation of cholecystokinin-(29-33)-amide in mouse brain is dependent on tripeptidyl peptidase-I: implications for the degradation and storage of peptides in classical late-infantile neuronal ceroid lipofuscinosis.Alterations in ROS activity and lysosomal pH account for distinct patterns of macroautophagy in LINCL and JNCL fibroblasts.Lysine 43 is trimethylated in subunit C from bovine mitochondrial ATP synthase and in storage bodies associated with batten disease.Determination of the substrate specificity of tripeptidyl-peptidase I using combinatorial peptide libraries and development of improved fluorogenic substrates.Neuronal loss and brain atrophy in mice lacking cathepsins B and LThe genetic spectrum of human neuronal ceroid-lipofuscinoses.Systemic administration of tripeptidyl peptidase I in a mouse model of late infantile neuronal ceroid lipofuscinosis: effect of glycan modification.Spectrum of ocular manifestations in CLN2-associated batten (Jansky-Bielschowsky) disease correlate with advancing age and deteriorating neurological functionPartial genetic suppression of a loss-of-function mutant of the neuronal ceroid lipofuscinosis-associated protease TPP1 in Dictyostelium discoideum.The neuronal ceroid-lipofuscinoses.Molecular background of progressive myoclonus epilepsy.When ethics constrains clinical research: trial design of control arms in "greater than minimal risk" pediatric trialsLarge-volume intrathecal enzyme delivery increases survival of a mouse model of late infantile neuronal ceroid lipofuscinosis.The intracellular location and function of proteins of neuronal ceroid lipofuscinoses.Gemfibrozil and fenofibrate, Food and Drug Administration-approved lipid-lowering drugs, up-regulate tripeptidyl-peptidase 1 in brain cells via peroxisome proliferator-activated receptor α: implications for late infantile Batten disease therapy.Brain Region-Specific Degeneration with Disease Progression in Late Infantile Neuronal Ceroid Lipofuscinosis (CLN2 Disease).Long-term expression and safety of administration of AAVrh.10hCLN2 to the brain of rats and nonhuman primates for the treatment of late infantile neuronal ceroid lipofuscinosis.Neuropeptide changes and neuroactive amino acids in CSF from humans and sheep with neuronal ceroid lipofuscinoses (NCLs, Batten disease).Analysis of NCL Proteins from an Evolutionary Standpoint.A mutation in the ovine cathepsin D gene causes a congenital lysosomal storage disease with profound neurodegeneration.Mannose 6-phosphorylated proteins are required for tumor necrosis factor-induced apoptosis: defective response in I-cell disease fibroblasts.A tripeptidyl peptidase 1 is a binding partner of the Golgi pH regulator (GPHR) in Dictyostelium.Proteomics Insights into Autophagy.Distribution of tripeptidyl peptidase I in human tissues under normal and pathological conditions.The specificity of lysosomal tripeptidyl peptidase-I determined by its action on angiotensin-II analogues.Diagnosis of late-infantile neuronal ceroid lipofuscinosis: a new sensitive method to assay lysosomal pepstatin-insensitive proteinase activity in human and animal specimens by capillary electrophoresis.Rat tripeptidyl peptidase I: molecular cloning, functional expression, tissue localization and enzymatic characterization.
P2860
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P2860
Classical late infantile neuronal ceroid lipofuscinosis fibroblasts are deficient in lysosomal tripeptidyl peptidase I
description
1999 nî lūn-bûn
@nan
1999 թուականի Յունուարին հրատարակուած գիտական յօդուած
@hyw
1999 թվականի հունվարին հրատարակված գիտական հոդված
@hy
1999年の論文
@ja
1999年論文
@yue
1999年論文
@zh-hant
1999年論文
@zh-hk
1999年論文
@zh-mo
1999年論文
@zh-tw
1999年论文
@wuu
name
Classical late infantile neuro ...... osomal tripeptidyl peptidase I
@ast
Classical late infantile neuro ...... osomal tripeptidyl peptidase I
@en
Classical late infantile neuro ...... osomal tripeptidyl peptidase I
@en-gb
Classical late infantile neuro ...... osomal tripeptidyl peptidase I
@nl
type
label
Classical late infantile neuro ...... osomal tripeptidyl peptidase I
@ast
Classical late infantile neuro ...... osomal tripeptidyl peptidase I
@en
Classical late infantile neuro ...... osomal tripeptidyl peptidase I
@en-gb
Classical late infantile neuro ...... osomal tripeptidyl peptidase I
@nl
prefLabel
Classical late infantile neuro ...... osomal tripeptidyl peptidase I
@ast
Classical late infantile neuro ...... osomal tripeptidyl peptidase I
@en
Classical late infantile neuro ...... osomal tripeptidyl peptidase I
@en-gb
Classical late infantile neuro ...... osomal tripeptidyl peptidase I
@nl
P2860
P921
P3181
P1433
P1476
Classical late infantile neuro ...... osomal tripeptidyl peptidase I
@en
P2093
M J Warburton
P2860
P3181
P356
10.1016/S0014-5793(98)01683-4
P407
P577
1999-01-25T00:00:00Z