Production and characterization of recombinant human CLN2 protein for enzyme-replacement therapy in late infantile neuronal ceroid lipofuscinosis (Q28367193)

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English	Production and characterization of recombinant human CLN2 protein for enzyme-replacement therapy in late infantile neuronal ceroid lipofuscinosis	scientific article

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scholarly article

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Production and characterization of recombinant human CLN2 protein for enzyme-replacement therapy in late infantile neuronal ceroid lipofuscinosis (English)

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Infantile neuronal ceroid lipofuscinosis

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enzyme replacement therapy

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Jansky–Bielschowsky disease

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based on heuristic

inferred from title

author name string

L Lin

1

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P Lobel

2

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language of work or name

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publication date

1 July 2001

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Biochemical Journal

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357

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Pt 1

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49-55

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Protein measurement with the Folin phenol reagent

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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1221927

20 March 2017

The human CLN2 protein/tripeptidyl-peptidase I is a serine protease that autoactivates at acidic pH

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1221927

20 March 2017

Purification and characterization of bovine brain lysosomal pepstatin-insensitive proteinase, the gene product deficient in the human late-infantile neuronal ceroid lipofuscinosis

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1221927

20 March 2017

Mutational analysis of the defective protease in classic late-infantile neuronal ceroid lipofuscinosis, a neurodegenerative lysosomal storage disorder

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1221927

20 March 2017

Classical late infantile neuronal ceroid lipofuscinosis fibroblasts are deficient in lysosomal tripeptidyl peptidase I

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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1221927

20 March 2017

Recombinant human sulphamidase: expression, amplification, purification and characterization

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20 March 2017

Association of mutations in a lysosomal protein with classical late-infantile neuronal ceroid lipofuscinosis

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1221927

20 March 2017

Purification and characterization of a tripeptidyl peptidase I from human osteoclastomas: evidence for its role in bone resorption

1 reference

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20 March 2017

Overexpression of human alpha-galactosidase A results in its intracellular aggregation, crystallization in lysosomes, and selective secretion

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20 March 2017

Proliferin secreted by cultured cells binds to mannose 6-phosphate receptors

1 reference

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20 March 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1221927

20 March 2017

Correction of glycogen storage disease type II by enzyme replacement with a recombinant human acid maltase produced by over-expression in a CHO-DHFR(neg) cell line

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29 September 2017

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29 September 2017

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29 September 2017

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29 September 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1221927

29 September 2017

Batten disease: four genes and still counting

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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1221927

29 September 2017

The neuronal ceroid-lipofuscinoses. Recent advances.

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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1221927

29 September 2017

Enzyme replacement therapy for Gaucher disease: critical investigations beyond demonstration of clinical efficacy

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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1221927

29 September 2017

Therapeutic response to intravenous infusions of glucocerebrosidase in a patient with Gaucher disease

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29 September 2017

Purification and characterisation of a tripeptidyl aminopeptidase I from rat spleen.

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https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1221927

2 June 2018

Overexpression of the human lysosomal enzyme alpha-L-iduronidase in Chinese hamster ovary cells

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Batten disease and the ATP synthase subunit c turnover pathway: raising antibodies to subunit c.

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Identification of a tripeptidyl aminopeptidase in the anterior pituitary gland: effect on the chemical and biological properties of rat and bovine growth hormones

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27 November 2018

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12 December 2020

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https://pubmed.ncbi.nlm.nih.gov/11415435

12 December 2020

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Human acid alpha-glucosidase from rabbit milk has therapeutic effect in mice with glycogen storage disease type II

1 reference

https://pubmed.ncbi.nlm.nih.gov/11415435

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Enzyme-based diagnosis of classical late infantile neuronal ceroid lipofuscinosis: comparison of tripeptidyl peptidase I and pepstatin-insensitive protease assays

1 reference

https://pubmed.ncbi.nlm.nih.gov/11415435

12 December 2020

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Partial purification and characterization of an ovarian tripeptidyl peptidase: a lysosomal exopeptidase that sequentially releases collagen-related (Gly-Pro-X) triplets

1 reference

https://pubmed.ncbi.nlm.nih.gov/11415435

12 December 2020

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Acidic tripeptidyl aminopeptidase in rat liver tritosomes: partial purification and determination of its primary substrate specificity

1 reference

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12 December 2020

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