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.
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)Moving towards effective therapeutic strategies for Neuronal Ceroid LipofuscinosisAAV gene transfer delays disease onset in a TPP1-deficient canine model of the late infantile form of Batten disease.Production 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 lipofuscinosisDetermination of the substrate specificity of tripeptidyl-peptidase I using combinatorial peptide libraries and development of improved fluorogenic substrates.Classification of subcellular location by comparative proteomic analysis of native and density-shifted lysosomesSedolisins, a new class of secreted proteases from Aspergillus fumigatus with endoprotease or tripeptidyl-peptidase activity at acidic pHs.Partial genetic suppression of a loss-of-function mutant of the neuronal ceroid lipofuscinosis-associated protease TPP1 in Dictyostelium discoideum.Regulation of chlamydial infection by host autophagy and vacuolar ATPase-bearing organelles.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.Different molecular mechanisms involved in spontaneous and oxidative stress-induced mitochondrial fragmentation in tripeptidyl peptidase-1 (TPP-1)-deficient fibroblasts.Lysosomal serine protease CLN2 regulates tumor necrosis factor-alpha-mediated apoptosis in a Bid-dependent mannerNeuronal ceroid lipofuscinosis type CLN2: a new rationale for the construction of phenotypic subgroups based on a survey of 25 cases in South America.Update of the mutation spectrum and clinical correlations of over 360 mutations in eight genes that underlie the neuronal ceroid lipofuscinoses.The role of nonsense-mediated decay in neuronal ceroid lipofuscinosis.A mutation in the ovine cathepsin D gene causes a congenital lysosomal storage disease with profound neurodegeneration.A novel role of the Batten disease gene CLN3: association with BMP synthesis.Distribution of tripeptidyl peptidase I in human tissues under normal and pathological conditions.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.Lysosomal peptidases and glycosidases in rheumatoid arthritis.Viral-mediated delivery of the late-infantile neuronal ceroid lipofuscinosis gene, TPP-I to the mouse central nervous system.Gemfibrozil, food and drug administration-approved lipid-lowering drug, increases longevity in mouse model of late infantile neuronal ceroid lipofuscinosis.Characterisation of lipofuscin-like lysosomal inclusion bodies from human placenta.
P2860
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P2860
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.
description
1999 nî lūn-bûn
@nan
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
1999年论文
@zh
1999年论文
@zh-cn
name
Biochemical characterization o ...... n specimens and animal models.
@en
type
label
Biochemical characterization o ...... n specimens and animal models.
@en
prefLabel
Biochemical characterization o ...... n specimens and animal models.
@en
P2093
P2860
P1476
Biochemical characterization o ...... n specimens and animal models.
@en
P2093
P2860
P304
P356
10.1046/J.1471-4159.1999.0730700.X
P407
P577
1999-08-01T00:00:00Z