Mouse "protective protein". cDNA cloning, sequence comparison, and expression.
Open accessAbstract
The "protective protein" is the glycoprotein that forms a complex with the lysosomal enzymes beta-galactosidase and neuraminidase. Its deficiency in man leads to the metabolic storage disorder galactosialidosis. The primary structure of human protective protein, deduced from its cloned cDNA, shows homology to yeast serine carboxypeptidases. We have isolated a full-length cDNA encoding murine protective protein. The nucleotide sequences as well as the predicted amino acid sequences are highly conserved between man and mouse. Domains important for the protease function are completely identical in the two proteins. Both human and mouse mature protective proteins covalently bind radiolabeled diisopropyl fluorophosphate. Transient expression of the murine cDNA in COS-1 cells yields a protective protein precursor of 54 kDa, a size characteristic of the glycosylated form. This cDNA-encoded precursor, endocytosed by human galactosialidosis fibroblasts, is processed into a 32- and a 20-kDa heterodimer and corrects beta-galactosidase and neuraminidase activities. A tissue-specific expression of protective protein mRNA is observed when total RNA from different mouse organs is analyzed on Northern blots.
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Mouse PPCA is 87% identical at the amino-acid level to human PPCA, shares similar biochemical and kinetic properties, and can substitute for the human enzyme in correcting both NEU1 and β-GAL activities when taken up by patients' fibroblasts.
“Mouse PPCA 32 is 87% identical at the amino-acid level to the human counterpart, and shares similar biochemical and kinetic properties.”
From Galactosialidosis: historic aspects and overview of investigated and emerging treatment options (2017) · cites this paper as [32]
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Galactosialidosis: historic aspects and overview of investigated and emerging treatment options
Annunziata, I., & d’Azzo, A. (2017) · Expert Opinion on Orphan Drugs · doi:10.1080/21678707.2016.1266933 · Cited by 50 · see citing works
Self-citation
- Authors
- Galjart, N. J., Gillemans, N., Meijer, D., & d'Azzo, A.
- Affiliations
- Erasmus University Rotterdam (NL)
- Venue
- Journal of Biological Chemistry
- Publisher
- Elsevier BV
- Published
- 1990-03
- Volume
- 265
- Issue
- 8
- Pages
- 4678-4684
- ISSN
- 0021-9258 (ISSN-L)
- OpenAlex
- W1515556278
- Cited by
- 76
- References
- 0
- Citations by year
- 2025: 1 · 2024: 1 · 2020: 2 · 2019: 1 · 2018: 2 · 2017: 2 · 2016: 2 · 2014: 1 · 2012: 1
- FWCI
- 5.23
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Licence
- Locations
- Journal of Biological Chemistry (publishedVersion, cc-by)
- PubMed (publishedVersion)
- EUR Research Repository (Erasmus University Rotterdam) (publishedVersion, cc-by)
- Topics
- Health Sciences › Medicine › Physiology › Lysosomal Storage Disorders Research
- Health Sciences › Medicine › Rheumatology › Glycogen Storage Diseases and Myoclonus
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Keywords
- protective proteins, lysosomal glycoproteins, galactosialidosis, beta-galactosidase, neuraminidase, cDNA cloning, mice, human-mouse sequence homology, proteolytic activity, glycosylation, endocytosis, heterodimer, tissue-specific mRNA expression, lysosomal storage disease
- MeSH terms
- Amino Acid Sequence, Animals, Base Sequence, beta-Galactosidase, Carboxypeptidases, Cell Line, Cloning, Molecular, DNA, Glycoproteins, Cricetinae, Humans, Isoflurophate, Lysosomes, Molecular Sequence Data, Neuraminidase, Nucleic Acid Hybridization, RNA, Messenger, Sequence Homology, Nucleic Acid, Tissue Distribution, Transfection, Restriction Mapping, Gene Expression, Cathepsin A, Mice
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