Plasma calprotectin is extremely high in patients with lysinuric protein intolerance
Abstract Lysinuric protein intolerance (LPI) is a rare autosomal recessive disorder affecting the transport of cationic amino acids. Elevated plasma zinc concentrations have been described in patients with LPI. Calprotectin is a calcium‐ and zinc‐binding protein, produced by polymorphonuclear leukoc...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2023-07-01
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| Series: | JIMD Reports |
| Subjects: | |
| Online Access: | https://doi.org/10.1002/jmd2.12377 |
| _version_ | 1797789295855009792 |
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| author | Mari Kärki Laura Tanner Satu Lahtinen Tero Soukka Harri Niinikoski |
| author_facet | Mari Kärki Laura Tanner Satu Lahtinen Tero Soukka Harri Niinikoski |
| author_sort | Mari Kärki |
| collection | DOAJ |
| description | Abstract Lysinuric protein intolerance (LPI) is a rare autosomal recessive disorder affecting the transport of cationic amino acids. Elevated plasma zinc concentrations have been described in patients with LPI. Calprotectin is a calcium‐ and zinc‐binding protein, produced by polymorphonuclear leukocytes and monocytes. Both zinc and calprotectin have an important role in immune system. In this study, we describe plasma zinc and plasma calprotectin concentrations in Finnish LPI patients. Plasma calprotectin concentration was measured from 10 LPI patients using an enzyme‐linked immunosorbent assay (ELISA) and it was remarkably high in all LPI patients (median: 622 338 μg/L) compared to that in healthy controls (608 μg/L). Plasma zinc concentration was measured by photometry and it was normal or only mildly elevated (median: 14.9 μmol/L). All the patients had decreased glomerular infiltration rate (median: 50 mL/min/1.73 m2). In conclusion, we observed extremely high plasma calprotectin concentration in patients with LPI. Mechanism of this phenomenon is unknown. |
| first_indexed | 2024-03-13T01:48:35Z |
| format | Article |
| id | doaj.art-890f64f6f748467793b90abbf969687e |
| institution | Directory Open Access Journal |
| issn | 2192-8312 |
| language | English |
| last_indexed | 2024-03-13T01:48:35Z |
| publishDate | 2023-07-01 |
| publisher | Wiley |
| record_format | Article |
| series | JIMD Reports |
| spelling | doaj.art-890f64f6f748467793b90abbf969687e2023-07-03T02:51:12ZengWileyJIMD Reports2192-83122023-07-0164429329910.1002/jmd2.12377Plasma calprotectin is extremely high in patients with lysinuric protein intoleranceMari Kärki0Laura Tanner1Satu Lahtinen2Tero Soukka3Harri Niinikoski4Department of Pediatrics University of Turku Turku FinlandDepartment of Clinical Genetics Helsinki University Hospital Helsinki FinlandDepartment of Life Technologies/Biotechnology University of Turku Turku FinlandDepartment of Life Technologies/Biotechnology University of Turku Turku FinlandInstitute of Biomedicine University of Turku Turku FinlandAbstract Lysinuric protein intolerance (LPI) is a rare autosomal recessive disorder affecting the transport of cationic amino acids. Elevated plasma zinc concentrations have been described in patients with LPI. Calprotectin is a calcium‐ and zinc‐binding protein, produced by polymorphonuclear leukocytes and monocytes. Both zinc and calprotectin have an important role in immune system. In this study, we describe plasma zinc and plasma calprotectin concentrations in Finnish LPI patients. Plasma calprotectin concentration was measured from 10 LPI patients using an enzyme‐linked immunosorbent assay (ELISA) and it was remarkably high in all LPI patients (median: 622 338 μg/L) compared to that in healthy controls (608 μg/L). Plasma zinc concentration was measured by photometry and it was normal or only mildly elevated (median: 14.9 μmol/L). All the patients had decreased glomerular infiltration rate (median: 50 mL/min/1.73 m2). In conclusion, we observed extremely high plasma calprotectin concentration in patients with LPI. Mechanism of this phenomenon is unknown.https://doi.org/10.1002/jmd2.12377Finnish disease heritagehypercalprotectinemiaLysinuric protein intolerancerenal insufficiency |
| spellingShingle | Mari Kärki Laura Tanner Satu Lahtinen Tero Soukka Harri Niinikoski Plasma calprotectin is extremely high in patients with lysinuric protein intolerance JIMD Reports Finnish disease heritage hypercalprotectinemia Lysinuric protein intolerance renal insufficiency |
| title | Plasma calprotectin is extremely high in patients with lysinuric protein intolerance |
| title_full | Plasma calprotectin is extremely high in patients with lysinuric protein intolerance |
| title_fullStr | Plasma calprotectin is extremely high in patients with lysinuric protein intolerance |
| title_full_unstemmed | Plasma calprotectin is extremely high in patients with lysinuric protein intolerance |
| title_short | Plasma calprotectin is extremely high in patients with lysinuric protein intolerance |
| title_sort | plasma calprotectin is extremely high in patients with lysinuric protein intolerance |
| topic | Finnish disease heritage hypercalprotectinemia Lysinuric protein intolerance renal insufficiency |
| url | https://doi.org/10.1002/jmd2.12377 |
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