(Q42854024)

8 November 2017

Impaired clearance of free cystine from lysosome-enriched granular fractions of I-cell-disease fibroblasts (English)

1 reference

8 November 2017

F Tietze

1

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8 November 2017

L H Rome

2

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8 November 2017

J D Butler

3

1 reference

8 November 2017

G S Harper

4

1 reference

8 November 2017

W A Gahl

5

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8 November 2017

language of work or name

English

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publication date

1 July 1986

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8 November 2017

7

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inferred from page(s)

published in

Biochemical Journal

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8 November 2017

237

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8 November 2017

1

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8 November 2017

9-15

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Protein measurement with the Folin phenol reagent

8 November 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1146941

Accumulation of weak bases in relation to intralysosomal pH in cultured human skin fibroblasts

27 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1146941

Cystine transport is defective in isolated leukocyte lysosomes from patients with cystinosis

27 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1146941

Defective catabolism of low-density lipoprotein by fibroblasts from patients with I-cell disease

27 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1146941

Biosynthesis of lysosomal enzymes in fibroblasts. Synthesis as precursors of higher molecular weight.

27 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1146941

Cystine accumulation and clearance by normal and cystinotic leukocytes exposed to cystine dimethyl ester

27 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1146941

Lysosomal cystine counter-transport in heterozygotes for cystinosis

27 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1146941

Characteristics of cystine counter-transport in normal and cystinotic lysosome-rich leucocyte granular fractions

27 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1146941

Lysosomal enzyme targeting. N-Acetylglucosaminylphosphotransferase selectively phosphorylates native lysosomal enzymes

27 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1146941

UDP-N-acetylglucosamine:glycoprotein N-acetylglucosamine-1-phosphotransferase. Proposed enzyme for the phosphorylation of the high mannose oligosaccharide units of lysosomal enzymes

27 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1146941

The Phosphomannosyl Recognition System for Intracellular and Intercellular Transport of Lysosomal Enzymes

27 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1146941

Fibroblasts from patients with I-cell disease and pseudo-Hurler polydystrophy are deficient in uridine 5'-diphosphate-N-acetylglucosamine: glycoprotein N-acetylglucosaminylphosphotransferase activity.

27 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1146941

I-cell disease: biochemical studies

27 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1146941

Detection and characterization of carrier-mediated cationic amino acid transport in lysosomes of normal and cystinotic human fibroblasts. Role in therapeutic cystine removal?

27 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1146941

pH effects on cystine transport in lysosome-rich leucocyte granular fractions

27 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1146941

Accumulation of amino acids by lysosomes incubated with amino acid methyl esters

27 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1146941

Two species of lysosomal organelles in cultured human fibroblasts

27 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1146941

Phosphohexosyl components of a lysosomal enzyme are recognized by pinocytosis receptors on human fibroblasts

27 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1146941

46 Enzymic diagnosis of the genetic mucopolysaccharide storage disorders

27 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1146941

ATP-dependent lysosomal cystine efflux is defective in cystinosis

27 May 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1146941

Deficiency of UDP-N-acetylglucosamine: Lysosomal enzyme N-acetylglucosamine-1-phosphotransferase in organs of I-cell patients

16 August 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1146941

Cystine: Compartmentalization within Lysosomes in Cystinotic Leukocytes

16 August 2018

1 reference

12 December 2020

Ultrastructure of Cultured Fibroblasts in I-Cell Disease

1 reference

12 December 2020

Rupture of rat liver lysosomes mediated by L-amino acid esters

1 reference

12 December 2020

Binding assays for amino acids. The utilization of a cystine binding protein from Escherichia coli for the determination of acid-soluble cystine in small physiological samples

1 reference

12 December 2020

I-cell disease: activities of lysosomal enzymes toward natural and synthetic substrates

1 reference

12 December 2020

Lysosomal cystine storage in cystinosis and mucolipidosis type II

1 reference

12 December 2020

Elevated Cystine Levels in Cultured Skin Fibroblasts From Patients With I-Cell Disease

1 reference

12 December 2020

Biosynthesis of lysosomal enzymes in fibroblasts. Phosphorylation of mannose residues

1 reference

12 December 2020

Enzymatic phosphorylation of lysosomal enzymes in the presence of UDP-N-acetylglucosamine. Absence of the activity in I-cell fibroblasts

1 reference

12 December 2020

Patterns of amino acid efflux from isolated normal and cystinotic human leucocyte lysosomes

1 reference

12 December 2020

Defective cystine exodus from isolated lysosome-rich fractions of cystinotic leucocytes

1 reference

12 December 2020

The Effect of Pertussis Vaccine on the Insulin-Dependent Diabetes Induced by Streptozotocin in Mice

1 reference

12 December 2020

Cystine accumulation and clearance in normal and cystinotic fibroblasts exposed to cystine dimethyl ester

1 reference

12 December 2020

Comparative study of cystine clearance in cystinotic and I-cell fibroblasts upon exposure to cystine dimethyl ester

1 reference

12 December 2020

Identifiers

DOI

10.1042/BJ2370009

1 reference

8 November 2017

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8 November 2017

PubMed publication ID

3800891

1 reference