(Q43117772)

14 November 2017

title

Functional consequences and rescue potential of pathogenic missense mutations in tripeptidyl peptidase I. (English)

1 reference

14 November 2017

Mariusz Walus

1

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14 November 2017

Elizabeth Kida

series ordinal

2

1 reference

14 November 2017

Adam A Golabek

series ordinal

3

1 reference

14 November 2017

publication date

1 June 2010

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14 November 2017

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14 November 2017

volume

31

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14 November 2017

issue

6

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14 November 2017

page(s)

710-721

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Mechanistic studies on the inactivation of the proteasome by lactacystin: a central role for clasto-lactacystin beta-lactone

14 November 2017

cites work

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

Tripeptidyl-peptidase I is apparently the CLN2 protein absent in classical late-infantile neuronal ceroid lipofuscinosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

Invited review: Effects of heat and cold stress on mammalian gene expression

21 January 2018

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Sorting of lysosomal proteins

21 January 2018

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7 January 2021

Chemical chaperones correct the mutant phenotype of the delta F508 cystic fibrosis transmembrane conductance regulator protein

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7 January 2021

Chemical chaperones mediate increased secretion of mutant alpha 1-antitrypsin (alpha 1-AT) Z: A potential pharmacological strategy for prevention of liver injury and emphysema in alpha 1-AT deficiency

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

Accelerated transport and maturation of lysosomal α–galactosidase A in Fabry lymphoblasts by an enzyme inhibitor

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

Mechanism of protein stabilization by glycerol: preferential hydration in glycerol-water mixtures

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

Low temperature restoring effect on F508del-CFTR misprocessing: A proteomic approach

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

SWISS-MODEL and the Swiss-PdbViewer: an environment for comparative protein modeling

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

Site-directed mutagenesis by overlap extension using the polymerase chain reaction

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

EDEM accelerates ERAD by preventing aberrant dimer formation of misfolded alpha1-antitrypsin

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

Protein oligomerization in the endoplasmic reticulum

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

Lectin-deficient calreticulin retains full functionality as a chaperone for class I histocompatibility molecules.

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

Identification of novel CLN2 mutations shows Canadian specific NCL2 alleles

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

Hypothermia after cardiac arrest

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

Cellular pathology and pathogenic aspects of neuronal ceroid lipofuscinoses

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

ConSurf 2005: the projection of evolutionary conservation scores of residues on protein structures

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

Expression and analysis of CLN2 variants in CHO cells: Q100R represents a polymorphism, and G389E and R447H represent loss-of-function mutations

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

N-linked glycan recognition and processing: the molecular basis of endoplasmic reticulum quality control

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

The recognition and retrotranslocation of misfolded proteins from the endoplasmic reticulum

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

Catalytic residues and substrate specificity of recombinant human tripeptidyl peptidase I (CLN2).

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

Contribution of a prosegment lysine residue to the function and structure of porcine pepsinogen A and its active form pepsin A

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

Endoplasmic reticulum-associated degradation

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

Protein N-glycosylation along the secretory pathway: relationship to organelle topography and function, protein quality control, and cell interactions

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

Chemical chaperones increase the cellular activity of N370S beta -glucosidase: a therapeutic strategy for Gaucher disease

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

Residual levels of tripeptidyl-peptidase I activity dramatically ameliorate disease in late-infantile neuronal ceroid lipofuscinosis

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

Late infantile neuronal ceroid lipofuscinosis: quantitative description of the clinical course in patients with CLN2 mutations

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

Mutations in classical late infantile neuronal ceroid lipofuscinosis disrupt transport of tripeptidyl-peptidase I to lysosomes

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

Therapeutic hypothermia for acute liver failure.

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

The proregion of cathepsin L is required for proper folding, stability, and ER exit

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

Effect of 35 degrees C hypothermia on intracranial pressure and clinical outcome in patients with severe traumatic brain injury

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

Tripeptidyl peptidases: enzymes that count

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

ERdj5 is required as a disulfide reductase for degradation of misfolded proteins in the ER

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

Mistargeting of the lectin ERGIC-53 to the endoplasmic reticulum of HeLa cells impairs the secretion of a lysosomal enzyme

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

An Adaptable Standard for Protein Export from the Endoplasmic Reticulum

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

Reevaluation of neuronal ceroid lipofuscinoses: atypical juvenile onset may be the result of CLN2 mutations.

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

Tripeptidyl-peptidase I in neuronal ceroid lipofuscinoses and other lysosomal storage disorders

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

A model of tripeptidyl-peptidase I (CLN2), a ubiquitous and highly conserved member of the sedolisin family of serine-carboxyl peptidases

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

Eris: an automated estimator of protein stability.

1 reference

https://api.crossref.org/works/10.1002%2FHUMU.21251

7 January 2021

Identifiers

DOI

10.1002/HUMU.21251

1 reference

14 November 2017

1 reference