Protective Protein as an Endogenous Endothelin Degradation Enzyme in Human Tissues (∗)
An enzyme hydrolyzing the carboxyl terminus of endothelin-1 was detected in control human tissues but was deficient in tissues from a patient with galactosialidosis, a metabolic disease caused by the protective protein gene mutation. It was proportional to the amount of immunologically estimated mat...
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| Veröffentlicht in: | The Journal of biological chemistry 1995-01, Vol.270 (2), p.515-518 |
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| container_issue | 2 |
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| container_title | The Journal of biological chemistry |
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| creator | Itoh, Kohji Kase, Ryoichi Shimmoto, Michie Satake, Akira Sakuraba, Hitoshi Suzuki, Yoshiyuki |
| description | An enzyme hydrolyzing the carboxyl terminus of endothelin-1 was detected in control human tissues but was deficient in tissues from a patient with galactosialidosis, a metabolic disease caused by the protective protein gene mutation. It was proportional to the amount of immunologically estimated mature protective protein. An antibody against the lysosomal protective protein/β-galactosidase complex precipitated the enzyme activity almost completely. Transfection of the human cDNA for protective protein resulted in high expression of the enzyme activity in transformed fibroblasts from a galactosialidosis patient. These results indicated that the mature protective protein is a major soluble endogenous endothelin degradation enzyme in human tissues. |
| doi_str_mv | 10.1074/jbc.270.2.515 |
| format | Article |
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| identifier | ISSN: 0021-9258 |
| ispartof | The Journal of biological chemistry, 1995-01, Vol.270 (2), p.515-518 |
| issn | 0021-9258 1083-351X |
| language | eng |
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| source | MEDLINE; EZB-FREE-00999 freely available EZB journals; Alma/SFX Local Collection |
| subjects | beta -galactosidase beta-Galactosidase - genetics beta-Galactosidase - metabolism Brain - enzymology Carboxypeptidases - genetics Carboxypeptidases - metabolism Cathepsin A DNA, Complementary endothelin-1 Endothelins - metabolism galactosialidosis Glycoproteins - genetics Glycoproteins - metabolism Humans Kidney - enzymology Liver - enzymology Lung - enzymology lysosomal protective protein Lysosomal Storage Diseases - enzymology man Precipitin Tests |
| title | Protective Protein as an Endogenous Endothelin Degradation Enzyme in Human Tissues (∗) |
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