Saposin C is required for normal resistance of acid beta-glucosidase to proteolytic degradation (Q44482194)

3 December 2017

Saposin C is required for normal resistance of acid beta-glucosidase to proteolytic degradation (English)

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3 December 2017

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GOA release 2020-03-11

author name string

Ying Sun

series ordinal

1

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3 December 2017

Xiaoyang Qi

series ordinal

2

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3 December 2017

Gregory A Grabowski

series ordinal

3

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3 December 2017

language of work or name

English

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publication date

17 June 2003

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Journal of Biological Chemistry

3 December 2017

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3 December 2017

278

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3 December 2017

34

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3 December 2017

31918-31923

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Structure and evolution of the human prosaposin chromosomal gene

3 December 2017

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https://api.crossref.org/works/10.1074%2FJBC.M302752200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

Biosynthesis, processing, and targeting of sphingolipid activator protein (SAP )precursor in cultured human fibroblasts. Mannose 6-phosphate receptor-independent endocytosis of SAP precursor

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

Proteolytic processing patterns of prosaposin in insect and mammalian cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

A novel mutation in the coding region of the prosaposin gene leads to a complete deficiency of prosaposin and saposins, and is associated with a complex sphingolipidosis dominated by lactosylceramide accumulation

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

Mutational analysis in a patient with a variant form of Gaucher disease caused by SAP-2 deficiency

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

Insertion in the mRNA of a metachromatic leukodystrophy patient with sphingolipid activator protein-1 deficiency

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

A mutation in the saposin A domain of the sphingolipid activator protein (prosaposin) gene results in a late-onset, chronic form of globoid cell leukodystrophy in the mouse

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

Targeted disruption of the mouse sphingolipid activator protein gene: a complex phenotype, including severe leukodystrophy and wide-spread storage of multiple sphingolipids.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

Acid beta-glucosidase: enzymology and molecular biology of Gaucher disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

Metabolism of glycolipids: the role of glycolipid-binding proteins in the function and pathobiochemistry of lysosomes

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

Crystal structure of saposin B reveals a dimeric shell for lipid binding

21 January 2018

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https://api.crossref.org/works/10.1074%2FJBC.M302752200

Differential membrane interactions of saposins A and C: implications for the functional specificity

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

Acid beta-glucosidase: intrinsic fluorescence and conformational changes induced by phospholipids and saposin C.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

Saposins (sap) A and C activate the degradation of galactosylceramide in living cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

Stimulation of acid ceramidase activity by saposin D.

21 January 2018

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https://api.crossref.org/works/10.1074%2FJBC.M302752200

Model SV40-transformed fibroblast lines for metabolic studies of human prosaposin and acid ceramidase deficiencies

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

Prosaposin: threshold rescue and analysis of the "neuritogenic" region in transgenic mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

Analyses of temporal regulatory elements of the prosaposin gene in transgenic mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

Sphingolipid metabolism. Sphingoid analogs, sphingolipid activator proteins, and the pathology of the cell.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

Association of N-acetylgalactosamine-6-sulfate sulfatase with the multienzyme lysosomal complex of beta-galactosidase, cathepsin A, and neuraminidase. Possible implication for intralysosomal catabolism of keratan sulfate

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

Transport of human lysosomal neuraminidase to mature lysosomes requires protective protein/cathepsin A.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

Mouse model of GM2 activator deficiency manifests cerebellar pathology and motor impairment

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

Saposin A: second cerebrosidase activator protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

Lysosomal degradation on vesicular membrane surfaces. Enhanced glucosylceramide degradation by lysosomal anionic lipids and activators.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

Activator proteins and topology of lysosomal sphingolipid catabolism.

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

Differential effects of proteinase inhibitors and amines on the lysosomal and non-lysosomal pathways of protein degradation in isolated rat hepatocytes

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

Fate and sorting of acid beta-glucosidase in transgenic mammalian cells

21 January 2018

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https://api.crossref.org/works/10.1074%2FJBC.M302752200

Saposins and their interaction with lipids

21 January 2018

1 reference

https://api.crossref.org/works/10.1074%2FJBC.M302752200

21 January 2018

Identifiers

DOI

10.1074/JBC.M302752200

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3 December 2017

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