(Q42071359)

English

Additional biochemical findings in a patient and fetal sibling with a genetic defect in the sphingolipid activator protein (SAP) precursor, prosaposin. Evidence for a deficiency in SAP-1 and for a normal lysosomal neuraminidase

scientific article published on July 15, 1992

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scholarly article

1 reference

Europe PubMed Central

16 October 2017

Additional biochemical findings in a patient and fetal sibling with a genetic defect in the sphingolipid activator protein (SAP) precursor, prosaposin. Evidence for a deficiency in SAP-1 and for a normal lysosomal neuraminidase (English)

1 reference

10.1042/BJ2850481

https://api.crossref.org/works/10.1042/BJ2850481

19 October 2022

1 reference

based on heuristic

inferred from title

1 reference

based on heuristic

inferred from title

1 reference

10.1042/BJ2850481

https://api.crossref.org/works/10.1042/BJ2850481

19 October 2022

1 reference

10.1042/BJ2850481

https://api.crossref.org/works/10.1042/BJ2850481

19 October 2022

author name string

Kustermann-Kuhn B

3

1 reference

Europe PubMed Central

16 October 2017

B. C. Paton

1

1 reference

Europe PubMed Central

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19 October 2022

B. Schmid

2

1 reference

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19 October 2022

B. Kustermann-Kuhn

3

1 reference

Europe PubMed Central

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19 October 2022

A. Poulos

4

1 reference

Europe PubMed Central

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A1637339

19 October 2022

K. Harzer

5

1 reference

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19 October 2022

language of work or name

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publication date

1 July 1992

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Europe PubMed Central

16 October 2017

15 July 1992

1 reference

National Center for Biotechnology Information

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19 October 2022

full work available at URL

https://www.ncbi.nlm.nih.gov/pmc/articles/pmid/1637339/?tool=EBI

online access status

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Europe PubMed Central

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19 October 2022

https://www.ncbi.nlm.nih.gov/pmc/articles/pmid/1637339/pdf/?tool=EBI

Portable Document Format

online access status

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19 October 2022

https://europepmc.org/articles/PMC1132813

online access status

content deliverer

Europe PubMed Central

1 reference

Europe PubMed Central

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A1637339

19 October 2022

https://europepmc.org/articles/PMC1132813?pdf=render

Portable Document Format

online access status

content deliverer

Europe PubMed Central

1 reference

Europe PubMed Central

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A1637339

19 October 2022

https://portlandpress.com/biochemj/article-pdf/285/2/481/607863/bj2850481.pdf

Portable Document Format

1 reference

10.1042/BJ2850481

https://api.crossref.org/works/10.1042/BJ2850481

19 October 2022

Biochemical Journal

1 reference

Europe PubMed Central

16 October 2017

285 ( Pt 2)

1 reference

Europe PubMed Central

16 October 2017

481-488

1 reference

Europe PubMed Central

16 October 2017

Protein measurement with the Folin phenol reagent

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Diagnosis of metachromatic leukodystrophy, Krabbe disease, and Farber disease after uptake of fatty acid-labeled cerebroside sulfate into cultured skin fibroblasts

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Cerebroside sulfatase activator deficiency induced metachromatic leukodystrophy

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

A cerebrosidesulfatase from swine kidney

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Gaucher's disease: deficiency of 'acid' -glucosidase and reconstitution of enzyme activity in vitro

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Hydrolysis of galactosylceramide is catalyzed by two genetically distinct acid beta-galactosidases.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Human placental sialidase: partial purification and characterization

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Identity of the activator proteins for the enzymatic hydrolysis of sulfatide, ganglioside GM1, and globotriaosylceramide

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Analysis of the multiple forms of Gaucher spleen sphingolipid activator protein 2.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Ganglioside GM1 metabolism in living human fibroblasts with beta-galactosidase deficiency

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Biosynthesis and molecular cloning of sulfated glycoprotein 1 secreted by rat Sertoli cells: sequence similarity with the 70-kilodalton precursor to sulfatide/GM1 activator

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Immunochemical characterization of two activator proteins stimulating enzymic sphingomyelin degradation in vitro. Absence of one of them in a human Gaucher disease variant

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Activator protein required for the enzymatic hydrolysis of cerebroside sulfate. Deficiency in urine of patients affected with cerebroside sulfatase activator deficiency and identity with activators for the enzymatic hydrolysis of GM1 ganglioside and

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Saposin D: a sphingomyelinase activator

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Coding of two sphingolipid activator proteins (SAP-1 and SAP-2) by same genetic locus

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Ganglioside GM3 sialidase activity in fibroblasts of normal individuals and of patients with sialidosis and mucolipidosis IV. Subcellular distribution and and some properties

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Clinical, pathological, and biochemical studies on an infantile case of sulfatide/GM1 activator protein deficiency

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Isolation of a cDNA encoding the human GM2 activator protein

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Saposin A: second cerebrosidase activator protein

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Sphingolipid hydrolase activator proteins and their precursors.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Sphingolipid activator protein deficiency in a 16-week-old atypical Gaucher disease patient and his fetal sibling: biochemical signs of combined sphingolipidoses

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Structure of full-length cDNA coding for sulfatide activator, a Co-beta-glucosidase and two other homologous proteins: two alternate forms of the sulfatide activator

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Recycling of glucosylceramide and sphingosine for the biosynthesis of gangliosides and sphingomyelin in rat liver

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

The complete amino-acid sequences of human ganglioside GM2 activator protein and cerebroside sulfate activator protein

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Human neuraminidase is a 60 kDa-processing product of prosaposin

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Immunocytochemical localization of sphingolipid activator protein 2 (SAP-2) in normal and SAP-deficient fibroblasts.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Mutation in the sphingolipid activator protein 2 in a patient with a variant of Gaucher disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Secretion of sphingolipid hydrolase activator precursor, prosaposin

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Sulfatide activator protein. Alternative splicing that generates three mRNAs and a newly found mutation responsible for a clinical disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

The organization of the gene for the human cerebroside sulfate activator protein

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Saposin proteins: structure, function, and role in human lysosomal storage disorders

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Human placental sialidase complex: characterization of the 60 kDa protein that cross-reacts with anti-saposin antibodies.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Glycosphingolipid specificity of the human sulfatide activator protein

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Simultaneous deficiency of sphingolipid activator proteins 1 and 2 is caused by a mutation in the initiation codon of their common gene

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Specificity of low molecular weight glycoprotein effector of lipid glycosidase

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Purification and Characterization of an Activator Protein for the Degradation of Glycolipids GM2and GA2by Hexosaminidase A

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

AB variant of infantile GM2 gangliosidosis: deficiency of a factor necessary for stimulation of hexosaminidase A-catalyzed degradation of ganglioside GM2 and glycolipid GA2

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

Metachromatic leukodystrophy without arylsulfatase A deficiency

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

The activator of cerebroside-sulphatase. A model of the activation

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

5 June 2018

A protein activator of galactosylceramide beta-galactosidase.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

17 August 2018

Studies on the activation of the enzymatic hydrolysis of sphingomyelin liposomes.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

17 August 2018

Presence of alpha-anomeric glycosidic configuration in the glycolipids accumulated in kidney with Fabry's disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1132813

17 August 2018

Sphingolipid activator protein 1 deficiency in metachromatic leucodystrophy with normal arylsulphatase A activity. A clinical, morphological, biochemical and immunological study

1 reference

https://pubmed.ncbi.nlm.nih.gov/1637339

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Human acid beta-glucosidase. Use of inhibitory and activating monoclonal antibodies to investigate the enzyme's catalytic mechanism and saposin A and C binding sites

1 reference

https://pubmed.ncbi.nlm.nih.gov/1637339

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Activator protein deficient Gaucher's disease. A second patient with the newly identified lipid storage disorder

1 reference

https://pubmed.ncbi.nlm.nih.gov/1637339

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The identification of lysosomal ganglioside sialidase in human cells

1 reference

https://pubmed.ncbi.nlm.nih.gov/1637339

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Further studies on the activation of glucocerebrosidase by a heat-stable factor from Gaucher spleen

1 reference

https://pubmed.ncbi.nlm.nih.gov/1637339

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Isolation of two forms of an activator protein for the enzymic sphingomyelin degradation from human Gaucher spleen

1 reference

https://pubmed.ncbi.nlm.nih.gov/1637339

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Biosynthesis of the sulfatide/GM1 activator protein (SAP-1) in control and mutant cultured skin fibroblasts

1 reference

https://pubmed.ncbi.nlm.nih.gov/1637339

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Immunocytochemical localization of sphingolipid activator protein-1, the sulfatide/GM1 ganglioside activator, to lysosomes in human liver and colon

1 reference

https://pubmed.ncbi.nlm.nih.gov/1637339

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

The precursor of sulfatide activator protein is processed to three different proteins

1 reference

https://pubmed.ncbi.nlm.nih.gov/1637339

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Characterization of a nonspecific activator protein for the enzymatic hydrolysis of glycolipids

1 reference

https://pubmed.ncbi.nlm.nih.gov/1637339

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Synthesis and processing of sphingolipid activator protein-2 (SAP-2) in cultured human fibroblasts

1 reference

https://pubmed.ncbi.nlm.nih.gov/1637339

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Galactosylceramide- and lactosylceramide-loading studies in cultured fibroblasts from normal individuals and patients with globoid cell leukodystrophy (Krabbe's disease) and GM1-gangliosidosis

1 reference

https://pubmed.ncbi.nlm.nih.gov/1637339

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Galactosylceramide and galactosylsphingosine loading studies in cultured skin fibroblasts in human and murine globoid cell leukodystrophy

1 reference

https://pubmed.ncbi.nlm.nih.gov/1637339

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Complexing of Glycolipids and Their Transfer between Membranes by the Activator Protein for Degradation of Lysosomal Ganglioside GM2

1 reference

https://pubmed.ncbi.nlm.nih.gov/1637339

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

A variant form of metachromatic leukodystrophy without arylsulfatase deficiency

1 reference

https://pubmed.ncbi.nlm.nih.gov/1637339

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Mechanism of activation of glucocerebrosidase by CO-β-glucosidase (glucosidase activator protein)

1 reference

https://pubmed.ncbi.nlm.nih.gov/1637339

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

beta-Glucosidase activator protein from bovine spleen ("coglucosidase")

1 reference

https://pubmed.ncbi.nlm.nih.gov/1637339

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Globoid cell leukodystrophy (Krabbe's disease). Metabolic studies with cultured fibroblasts

1 reference

https://pubmed.ncbi.nlm.nih.gov/1637339

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.1042/BJ2850481

1 reference

Europe PubMed Central

16 October 2017

1 reference

Europe PubMed Central

16 October 2017

1 reference

Europe PubMed Central

16 October 2017

ResearchGate publication ID

0 references

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