(Q28475003)

English

A Critical Tryptophan and Ca2+ in Activation and Catalysis of TPPI, the Enzyme Deficient in Classic Late-Infantile Neuronal Ceroid Lipofuscinosis

scientific article published on August 3, 2010

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scholarly article

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A critical tryptophan and Ca2+ in activation and catalysis of TPPI, the enzyme deficient in classic late-infantile neuronal ceroid lipofuscinosis (English)

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A Critical Tryptophan and Ca2+ in Activation and Catalysis of TPPI, the Enzyme Deficient in Classic Late-Infantile Neuronal Ceroid Lipofuscinosis (English)

1 reference

10.1371/JOURNAL.PONE.0011929

https://api.crossref.org/works/10.1371/JOURNAL.PONE.0011929

17 June 2024

Infantile neuronal ceroid lipofuscinosis

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author name string

Salomon Kuizon

1

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Kathleen DiMaiuta

2

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Marius Walus

3

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Marisol Kuizon

5

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Elizabeth Kida

6

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Edmund C. Jenkins

4

0000-0002-6448-090X

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A20689811

17 June 2024

Adam A. Golabek

7

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A20689811

17 June 2024

Daniel O. Espinoza

8

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A20689811

17 June 2024

Raju K. Pullarkat

9

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A20689811

17 June 2024

Mohammed A. Junaid

10

1 reference

Europe PubMed Central

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https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A20689811

17 June 2024

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3 August 2010

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full work available at URL

https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0011929&type=printable

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1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A20689811

17 June 2024

https://www.ncbi.nlm.nih.gov/pmc/articles/pmid/20689811/?tool=EBI

online access status

content deliverer

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A20689811

17 June 2024

https://www.ncbi.nlm.nih.gov/pmc/articles/pmid/20689811/pdf/?tool=EBI

Portable Document Format

online access status

content deliverer

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A20689811

17 June 2024

https://europepmc.org/articles/PMC2914745

online access status

content deliverer

Europe PubMed Central

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A20689811

17 June 2024

https://europepmc.org/articles/PMC2914745?pdf=render

Portable Document Format

online access status

content deliverer

Europe PubMed Central

1 reference

Europe PubMed Central

PubMed publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A20689811

17 June 2024

http://dx.plos.org/10.1371/journal.pone.0011929

1 reference

10.1371/JOURNAL.PONE.0011929

https://api.crossref.org/works/10.1371/JOURNAL.PONE.0011929

17 June 2024

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5

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8

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e11929

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copyright license

Creative Commons Attribution 4.0 International

3 August 2010

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April 2022 Public Data File from Crossref

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Crystal Structure and Autoactivation Pathway of the Precursor Form of Human Tripeptidyl-peptidase 1, the Enzyme Deficient in Late Infantile Ceroid Lipofuscinosis

1 reference

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19 March 2017

Structure of tripeptidyl-peptidase I provides insight into the molecular basis of late infantile neuronal ceroid lipofuscinosis

1 reference

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19 March 2017

Protein measurement with the Folin phenol reagent

1 reference

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19 March 2017

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

1 reference

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19 March 2017

Characterization of endopeptidase activity of tripeptidyl peptidase-I/CLN2 protein which is deficient in classical late infantile neuronal ceroid lipofuscinosis

1 reference

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19 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

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19 March 2017

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

1 reference

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19 March 2017

Tripeptidyl-peptidase I is apparently the CLN2 protein absent in 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=2914745

19 March 2017

Structural organization and sequence of CLN2, the defective gene in classical late infantile neuronal ceroid lipofuscinosis

1 reference

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19 March 2017

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

1 reference

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19 March 2017

Calcium-independent subtilisin by design

1 reference

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19 March 2017

Targeted disruption of the Cln3 gene provides a mouse model for Batten disease. The Batten Mouse Model Consortium [corrected]

1 reference

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19 March 2017

Lysosomal serine protease CLN2 regulates tumor necrosis factor-alpha-mediated apoptosis in a Bid-dependent manner

1 reference

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29 September 2017

Treatment of late infantile neuronal ceroid lipofuscinosis by CNS administration of a serotype 2 adeno-associated virus expressing CLN2 cDNA.

1 reference

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29 September 2017

Prosegment of tripeptidyl peptidase I is a potent, slow-binding inhibitor of its cognate enzyme

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2914745

29 September 2017

Residual levels of tripeptidyl-peptidase I activity dramatically ameliorate disease in 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=2914745

29 September 2017

Atypical CLN2 with later onset and prolonged course: a neuropathologic study showing different sensitivity of neuronal subpopulations to TPP1 deficiency.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2914745

29 September 2017

Tripeptidyl-peptidase I in health and disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2914745

29 September 2017

Overexpression in colorectal carcinoma of two lysosomal enzymes, CLN2 and CLN1, involved in neuronal ceroid lipofuscinosis

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2914745

29 September 2017

Determination of the substrate specificity of tripeptidyl-peptidase I using combinatorial peptide libraries and development of improved fluorogenic substrates

1 reference

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29 September 2017

Correlations between genotype, ultrastructural morphology and clinical phenotype in the neuronal ceroid lipofuscinoses

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2914745

29 September 2017

The genetic spectrum of human neuronal ceroid-lipofuscinoses

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2914745

29 September 2017

Mutation of the glycosylated asparagine residue 286 in human CLN2 protein results in loss of enzymatic activity.

1 reference

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29 September 2017

N-glycosylation is crucial for folding, trafficking, and stability of human tripeptidyl-peptidase I.

1 reference

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Therapeutic approaches to protein-misfolding diseases

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Biosynthesis, glycosylation, and enzymatic processing in vivo of human tripeptidyl-peptidase I.

1 reference

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29 September 2017

Biochemistry of neuronal ceroid lipofuscinoses

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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

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29 September 2017

Specific alterations in levels of mannose 6-phosphorylated glycoproteins in different neuronal ceroid lipofuscinoses

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Hydrogen bond stereochemistry in protein structure and function

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Purification and characterisation of a tripeptidyl aminopeptidase I from rat spleen

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Follow-up study of subunit c of mitochondrial ATP synthase (SCMAS) in Batten disease and in unrelated lysosomal disorders.

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Maturation of the trans-Golgi network protease furin: compartmentalization of propeptide removal, substrate cleavage, and COOH-terminal truncation

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Intracranial delivery of CLN2 reduces brain pathology in a mouse model of classical late infantile neuronal ceroid lipofuscinosis.

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Intraventricular enzyme replacement improves disease phenotypes in a mouse model of late infantile neuronal ceroid lipofuscinosis

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Distribution of tripeptidyl peptidase I in human tissues under normal and pathological conditions

1 reference

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Ser475, Glu272, Asp276, Asp327, and Asp360 are involved in catalytic activity of human tripeptidyl-peptidase I.

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Mitochondrial ATP synthase subunit c storage in the ceroid-lipofuscinoses (Batten disease)

1 reference

https://pubmed.ncbi.nlm.nih.gov/20689811

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Molecular diagnosis of and carrier screening for the neuronal ceroid lipofuscinoses

1 reference

https://pubmed.ncbi.nlm.nih.gov/20689811

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Rapid immunologic diagnosis of classic late infantile neuronal ceroid lipofuscinosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/20689811

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

First-trimester diagnosis of late-infantile neuronal ceroid lipofuscinosis (LINCL) by tripeptidyl peptidase I assay and CLN2 mutation analysis

1 reference

https://pubmed.ncbi.nlm.nih.gov/20689811

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

MEROPS: the peptidase database

1 reference

https://pubmed.ncbi.nlm.nih.gov/20689811

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Exclusion of late infantile neuronal ceroid lipofuscinosis (LINCL) in a fetus by assay of tripeptidyl peptidase I in chorionic villi

1 reference

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inferred from PubMed ID database lookup

Identifiers

10.1371/JOURNAL.PONE.0011929

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2010PLoSO...511929K

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