Rat brain contains high levels of mannose-6-phosphorylated glycoproteins including lysosomal enzymes and palmitoyl-protein thioesterase, an enzyme implicated in infantile neuronal lipofuscinosis - Wikidata - awgldk - TriplyDB

Rat brain contains high levels of mannose-6-phosphorylated glycoproteins including lysosomal enzymes and palmitoyl-protein thioesterase, an enzyme implicated in infantile neuronal lipofuscinosis

Rat brain contains high levels of mannose-6-phosphorylated glycoproteins including lysosomal enzymes and palmitoyl-protein thioesterase, an enzyme implicated in infantile neuronal lipofuscinosis

http://www.wikidata.org/entity/Q28570730

about

The human CLN2 protein/tripeptidyl-peptidase I is a serine protease that autoactivates at acidic pH Identification of sites of mannose 6-phosphorylation on lysosomal proteins The proteome of lysosomes Lipid thioesters derived from acylated proteins accumulate in infantile neuronal ceroid lipofuscinosis: correction of the defect in lymphoblasts by recombinant palmitoyl-protein thioesterase Molecular cloning and expression of palmitoyl-protein thioesterase 2 (PPT2), a homolog of lysosomal palmitoyl-protein thioesterase with a distinct substrate specificity Functions of the alpha, beta, and gamma subunits of UDP-GlcNAc:lysosomal enzyme N-acetylglucosamine-1-phosphotransferase Mutational analysis of the defective protease in classic late-infantile neuronal ceroid lipofuscinosis, a neurodegenerative lysosomal storage disorder Evaluation of AAV-mediated Gene Therapy for Central Nervous System Disease in Canine Mucopolysaccharidosis VII.Structural basis for the insensitivity of a serine enzyme (palmitoyl-protein thioesterase) to phenylmethylsulfonyl fluoride Interactions of the proteins of neuronal ceroid lipofuscinosis: clues to function Production and characterization of recombinant human CLN2 protein for enzyme-replacement therapy in late infantile neuronal ceroid lipofuscinosis Loss of Niemann-Pick C1 or C2 protein results in similar biochemical changes suggesting that these proteins function in a common lysosomal pathway Murine UDP-GlcNAc:lysosomal enzyme N-acetylglucosamine-1-phosphotransferase lacking the gamma-subunit retains substantial activity toward acid hydrolases Intracellular localization of p40, a protein identified in a preparation of lysosomal membranes Biochemical characterization and lysosomal localization of the mannose-6-phosphate protein p76 (hypothetical protein LOC196463)Demonstration of lysosomal localization for the mammalian ependymin-related protein using classical approaches combined with a novel density shift method Towards a human repertoire of monocytic lysosomal proteins.Proteomic analysis of lysosomal acid hydrolases secreted by osteoclasts: implications for lytic enzyme transport and bone metabolism.Impaired lysosomal trimming of N-linked oligosaccharides leads to hyperglycosylation of native lysosomal proteins in mice with alpha-mannosidosis.Classification of subcellular location by comparative proteomic analysis of native and density-shifted lysosomes Glial fibrillary acidic protein is elevated in the lysosomal storage disease classical late-infantile neuronal ceroid lipofuscinosis, but is not a component of the storage material.Potential pitfalls and solutions for use of fluorescent fusion proteins to study the lysosome The intracellular location and function of proteins of neuronal ceroid lipofuscinoses.Endocytosis provides a major alternative pathway for lysosomal biogenesis in kidney proximal tubular cells.Significance and prognostic value of lysosomal enzyme activities measured in surgically operated adenocarcinomas of the gastroesophageal junction and squamous cell carcinomas of the lower third of esophagus.Reversible Cysteine Acylation Regulates the Activity of Human Palmitoyl-Protein Thioesterase 1 (PPT1).Thematic review series: lipid posttranslational modifications. Lysosomal metabolism of lipid-modified proteins.Residual levels of tripeptidyl-peptidase I activity dramatically ameliorate disease in late-infantile neuronal ceroid lipofuscinosis.Proteomic analysis of mouse models of Niemann-Pick C disease reveals alterations in the steady-state levels of lysosomal proteins within the brain.Acid phosphatase 5 is responsible for removing the mannose 6-phosphate recognition marker from lysosomal proteins.Extending the mannose 6-phosphate glycoproteome by high resolution/accuracy mass spectrometry analysis of control and acid phosphatase 5-deficient mice Proteomics of the lysosome.Mass spectrometry-based protein profiling to determine the cause of lysosomal storage diseases of unknown etiology 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.Enzyme-specific differences in mannose phosphorylation between GlcNAc-1-phosphotransferase αβ and γ subunit deficient zebrafish support cathepsin proteases as early mediators of mucolipidosis pathology.Altered glutamate receptor function in the cerebellum of the Ppt1-/- mouse, a murine model of infantile neuronal ceroid lipofuscinosis A mutation in the ovine cathepsin D gene causes a congenital lysosomal storage disease with profound neurodegeneration.Specific alterations in levels of mannose 6-phosphorylated glycoproteins in different neuronal ceroid lipofuscinoses.The mannose 6-phosphate glycoprotein proteome.alpha-Glucosidase and N-acetylglucosamine-6-sulphatase are the major mannose-6-phosphate glycoproteins in human urine.

P2860

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P2860

Rat brain contains high levels of mannose-6-phosphorylated glycoproteins including lysosomal enzymes and palmitoyl-protein thioesterase, an enzyme implicated in infantile neuronal lipofuscinosis

http://www.wikidata.org/entity/Q28570730

description

1996 թուականի Օգոստոսին հրատարակուած գիտական յօդուած

@hyw

1996 թվականի օգոստոսին հրատարակված գիտական հոդված

@hy

article publié dans la revue scientifique Journal of Biological Chemistry

@fr

artículu científicu espublizáu en 1996

@ast

im August 1996 veröffentlichter wissenschaftlicher Artikel

@de

scientific journal article

@en

vedecký článok (publikovaný 1996/08/09)

@sk

vědecký článek publikovaný v roce 1996

@cs

wetenschappelijk artikel (gepubliceerd op 1996/08/09)

@nl

наукова стаття, опублікована в серпні 1996

@uk

name

Rat brain contains high levels ...... antile neuronal lipofuscinosis

@ast

Rat brain contains high levels ...... antile neuronal lipofuscinosis

@en

Rat brain contains high levels ...... antile neuronal lipofuscinosis

@nl

type

label

Rat brain contains high levels ...... antile neuronal lipofuscinosis

@ast

Rat brain contains high levels ...... antile neuronal lipofuscinosis

@en

Rat brain contains high levels ...... antile neuronal lipofuscinosis

@nl

prefLabel

Rat brain contains high levels ...... antile neuronal lipofuscinosis

@ast

Rat brain contains high levels ...... antile neuronal lipofuscinosis

@en

Rat brain contains high levels ...... antile neuronal lipofuscinosis

@nl

P1433

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P2860

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P31

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P5875

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P921

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Q28570730-DF36DA67-8CD6-4937-AE67-665416AA150F

P356

JBC.271.32.19191

P1433

Journal of Biological Chemistry

P1476

Rat brain contains high levels ...... antile neuronal lipofuscinosis

@en

P2093

D. E. Sleat

http://www.w3.org/2001/XMLSchema#string

H. Lackland

http://www.w3.org/2001/XMLSchema#string

I. Sohar

http://www.w3.org/2001/XMLSchema#string

J. Majercak

http://www.w3.org/2001/XMLSchema#string

P. Lobel

http://www.w3.org/2001/XMLSchema#string

P2860

Expression of cDNA encoding the human "protective protein" associated with lysosomal beta-galactosidase and neuraminidase: homology to yeast proteases

Mutations in the palmitoyl protein thioesterase gene causing infantile neuronal ceroid lipofuscinosis

Cloning and sequence analysis of cDNA for human cathepsin D

Cleavage of structural proteins during the assembly of the head of bacteriophage T4

Proteolytic processing of human lysosomal arylsulfatase A

Cathepsin B, Cathepsin H, and cathepsin L

Signal sequence and DNA-mediated expression of human lysosomal alpha-galactosidase A

Structure and function of the mannose 6-phosphate/insulinlike growth factor II receptors.

Sixty-nine kilobases of contiguous human genomic sequence containing the alpha-galactosidase A and Bruton's tyrosine kinase loci.

Cloning, sequence and expression of rat cathepsin D.

P304

19191–19198

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P31

scholarly article

P356

10.1074/JBC.271.32.19191

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P407

P433

32

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P478

271

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P577

1996-08-09T00:00:00Z

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P5875

14498945

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P698

8702598

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P921

phosphorylation