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rdf:type |
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lifeskim:mentions |
umls-concept:C0005220,
umls-concept:C0006556,
umls-concept:C0017262,
umls-concept:C0027803,
umls-concept:C0030940,
umls-concept:C0033684,
umls-concept:C0043393,
umls-concept:C0086418,
umls-concept:C0185117,
umls-concept:C0332281,
umls-concept:C0679058,
umls-concept:C1334043,
umls-concept:C1547699,
umls-concept:C2697616,
umls-concept:C2700640,
umls-concept:C2911684
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pubmed:issue |
6
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pubmed:dateCreated |
1988-10-11
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pubmed:databankReference |
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pubmed:abstractText |
The "protective protein" is a glycoprotein that associates with lysosomal beta-galactosidase and neuraminidase and is deficient in the autosomal recessive disorder galactosialidosis. We have isolated the cDNA encoding human "protective protein". The clone recognizes a 2 kb mRNA in normal cells that is not evident in fibroblasts of an early infantile galactosialidosis patient. The cDNA directs the synthesis of a 452 amino acid precursor molecule that is processed in vivo to yield mature "protective protein," a heterodimer of 32 kd and 20 kd polypeptides held together by disulfide bridges. This mature form is also biologically functional since it restores beta-galactosidase and neuraminidase activities in galactosialidosis cells. The predicted amino acid sequence of the "protective protein" bears homology to yeast carboxypeptidase Y and the KEX1 gene product. This suggests a protease activity for the "protective protein."
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
IM
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pubmed:chemical |
http://linkedlifedata.com/resource/pubmed/chemical/CTSA protein, human,
http://linkedlifedata.com/resource/pubmed/chemical/Carboxypeptidases,
http://linkedlifedata.com/resource/pubmed/chemical/Cathepsin A,
http://linkedlifedata.com/resource/pubmed/chemical/DNA,
http://linkedlifedata.com/resource/pubmed/chemical/Galactosidases,
http://linkedlifedata.com/resource/pubmed/chemical/Glycoproteins,
http://linkedlifedata.com/resource/pubmed/chemical/Neuraminidase,
http://linkedlifedata.com/resource/pubmed/chemical/Peptide Hydrolases,
http://linkedlifedata.com/resource/pubmed/chemical/RNA, Messenger,
http://linkedlifedata.com/resource/pubmed/chemical/beta-Galactosidase,
http://linkedlifedata.com/resource/pubmed/chemical/serine carboxypeptidase
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pubmed:status |
MEDLINE
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pubmed:month |
Sep
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pubmed:issn |
0092-8674
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pubmed:author |
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pubmed:issnType |
Print
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pubmed:day |
9
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pubmed:volume |
54
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
755-64
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pubmed:dateRevised |
2009-11-19
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pubmed:meshHeading |
pubmed-meshheading:3136930-Amino Acid Sequence,
pubmed-meshheading:3136930-Base Sequence,
pubmed-meshheading:3136930-Carbohydrate Metabolism, Inborn Errors,
pubmed-meshheading:3136930-Carboxypeptidases,
pubmed-meshheading:3136930-Cathepsin A,
pubmed-meshheading:3136930-Cloning, Molecular,
pubmed-meshheading:3136930-DNA,
pubmed-meshheading:3136930-Galactosidases,
pubmed-meshheading:3136930-Gene Expression Regulation,
pubmed-meshheading:3136930-Glycoproteins,
pubmed-meshheading:3136930-Humans,
pubmed-meshheading:3136930-Lysosomes,
pubmed-meshheading:3136930-Molecular Sequence Data,
pubmed-meshheading:3136930-Neuraminidase,
pubmed-meshheading:3136930-Nucleic Acid Hybridization,
pubmed-meshheading:3136930-Peptide Hydrolases,
pubmed-meshheading:3136930-Protein Binding,
pubmed-meshheading:3136930-RNA, Messenger,
pubmed-meshheading:3136930-beta-Galactosidase
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pubmed:year |
1988
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pubmed:articleTitle |
Expression of cDNA encoding the human "protective protein" associated with lysosomal beta-galactosidase and neuraminidase: homology to yeast proteases.
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pubmed:affiliation |
Department of Cell Biology and Genetics, Erasmus University, Rotterdam, The Netherlands.
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pubmed:publicationType |
Journal Article,
Comparative Study
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