(Q30429249)

4 September 2017

The atomic model of the human protective protein/cathepsin A suggests a structural basis for galactosialidosis (English)

1 reference

4 September 2017

1 reference

G Rudenko

1

1 reference

4 September 2017

E Bonten

series ordinal

2

1 reference

4 September 2017

W G Hol

series ordinal

3

1 reference

4 September 2017

A d'Azzo

series ordinal

4

1 reference

4 September 2017

language of work or name

English

0 references

publication date

1 January 1998

1 reference

Proceedings of the National Academy of Sciences of the United States of America

4 September 2017

1 reference

4 September 2017

95

1 reference

4 September 2017

2

1 reference

4 September 2017

621-625

1 reference

The CCP4 suite: programs for protein crystallography

4 September 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

Raster3D Version 2.0. A program for photorealistic molecular graphics

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

Protein quality control: triage by chaperones and proteases

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

Cathepsin A deficiency in galactosialidosis: studies of patients and carriers in 16 families

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

Three-dimensional structure of the human 'protective protein': structure of the precursor form suggests a complex activation mechanism

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

Mutations in the catalytic domain of factor IX that are related to the subclass hemophilia Bm.

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

Protective protein gene mutations in galactosialidosis

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

Structural and genetic analysis of protein stability

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

Disease-causing mutations in exon 11 of the medium-chain acyl-CoA dehydrogenase gene

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

Salt bridge relay triggers defective LDL receptor binding by a mutant apolipoprotein

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

Dominant chymotrypsin-like esterase activity in human lymphocyte granules is mediated by the serine carboxypeptidase called cathepsin A-like protective protein

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

The molecular genetics of familial venous thrombosis

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

What do dysfunctional serpins tell us about molecular mobility and disease?

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

Structural interpretation of the mutations in the beta-cardiac myosin that have been implicated in familial hypertrophic cardiomyopathy

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

2.8-A structure of yeast serine carboxypeptidase

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

Lysosomal protective protein/cathepsin A. Role of the "linker" domain in catalytic activation

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

Quality control in the secretory pathway

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

Molecular defect in combined beta-galactosidase and neuraminidase deficiency in man.

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

Human placental neuraminidase. Activation, stabilization and association with beta-galactosidase and its protective protein

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

A factor IX mutation, verified by direct genomic sequencing, causes haemophilia B by a novel mechanism

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

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

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

Mouse "protective protein". cDNA cloning, sequence comparison, and expression

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

Three-dimensional structure of the LDL receptor-binding domain of human apolipoprotein E

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

Improved methods for building protein models in electron density maps and the location of errors in these models

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

Human lysosomal protective protein has cathepsin A-like activity distinct from its protective function

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

Lysosomal storage diseases

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

A mutation in a mild form of galactosialidosis impairs dimerization of the protective protein and renders it unstable

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

Inactivation of endothelin I by deamidase (lysosomal protective protein)

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

A peptidase in human platelets that deamidates tachykinins. Probable identity with the lysosomal "protective protein"

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

Refined atomic model of wheat serine carboxypeptidase II at 2.2-A resolution

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

Human lysosomal protective protein. Glycosylation, intracellular transport, and association with beta-galactosidase in the endoplasmic reticulum

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

Mutations in the medium chain acyl-CoA dehydrogenase (MCAD) gene

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

Macular cherry-red spots and myoclonus with dementia: Coexistent neuraminidase and β-galactosidase deficiencies

20 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

MOLSCRIPT: a program to produce both detailed and schematic plots of protein structures

20 June 2018

1 reference

https://api.crossref.org/works/10.1073%2FPNAS.95.2.621

Volume changes on protein folding.

21 January 2018

1 reference

https://api.crossref.org/works/10.1073%2FPNAS.95.2.621

Characterization of a factor IX variant with a glycine207 to glutamic acid mutation.

21 January 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=18470

Purification of acid beta-galactosidase and acid neuraminidase from bovine testis: evidence for an enzyme complex

26 November 2018

1 reference

https://api.crossref.org/works/10.1073%2FPNAS.95.2.621

Molecular and biochemical analysis of protective protein/cathepsin A mutations: correlation with clinical severity in galactosialidosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1073%2FPNAS.95.2.621

Homologous modeling of the lysosomal protective protein/carboxypeptidase L: structural and functional implications of mutations identified in galactosialidosis patients

21 January 2018

1 reference

https://api.crossref.org/works/10.1073%2FPNAS.95.2.621

The unstable haemoglobins

21 January 2018

1 reference

https://api.crossref.org/works/10.1073%2FPNAS.95.2.621

Can proteins be turned inside-out?

21 January 2018

1 reference

https://api.crossref.org/works/10.1073%2FPNAS.95.2.621

21 January 2018

Identifiers

DOI

10.1073/PNAS.95.2.621

1 reference

4 September 2017

0 references

1 reference

4 September 2017

PubMed publication ID

9435242

1 reference