Plasma Calprotectin is Extremely High in Patients with Lysinuric Protein Intolerance

Overview

Journal JIMD Rep

Publisher Wiley

Date 2023 Jul 5

PMID 37404678

Authors

Mari Karki

Laura Tanner

Satu Lahtinen

Tero Soukka

Harri Niinikoski

Affiliations

Soon will be listed here.

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 m). In conclusion, we observed extremely high plasma calprotectin concentration in patients with LPI. Mechanism of this phenomenon is unknown.

Citing Articles

Plasma calprotectin is extremely high in patients with lysinuric protein intolerance.

Karki M, Tanner L, Lahtinen S, Soukka T, Niinikoski H JIMD Rep. 2023; 64(4):293-299.

PMID: 37404678 PMC: 10315390. DOI: 10.1002/jmd2.12377.

References

Mannucci L, Emma F, Markert M, Bachmann C, Boulat O, Carrozzo R . Increased NO production in lysinuric protein intolerance. J Inherit Metab Dis. 2005; 28(2):123-9. DOI: 10.1007/s10545-005-5954-x. View

Malickova K, Brodska H, Lachmanova J, Dusilova Sulkova S, Janatkova I, Mareckova H . Plasma calprotectin in chronically dialyzed end-stage renal disease patients. Inflamm Res. 2009; 59(4):299-305. DOI: 10.1007/s00011-009-0103-x. View

de Baulny H, Schiff M, Dionisi-Vici C . Lysinuric protein intolerance (LPI): a multi organ disease by far more complex than a classic urea cycle disorder. Mol Genet Metab. 2012; 106(1):12-7. DOI: 10.1016/j.ymgme.2012.02.010. View

Chen J, Fan P, Kou G, Chang S, Chen Y, Lee C . Meta-Analysis: Urinary Calprotectin for Discrimination of Intrinsic and Prerenal Acute Kidney Injury. J Clin Med. 2019; 8(1). PMC: 6352097. DOI: 10.3390/jcm8010074. View

Pepper R, Wang H, Rajakaruna G, Papakrivopoulou E, Vogl T, Pusey C . S100A8/A9 (calprotectin) is critical for development of glomerulonephritis and promotes inflammatory leukocyte-renal cell interactions. Am J Pathol. 2015; 185(5):1264-74. PMC: 4419283. DOI: 10.1016/j.ajpath.2015.01.015. View

Dale I, Brandtzaeg P, Fagerhol M, Scott H . Distribution of a new myelomonocytic antigen (L1) in human peripheral blood leukocytes. Immunofluorescence and immunoperoxidase staining features in comparison with lysozyme and lactoferrin. Am J Clin Pathol. 1985; 84(1):24-34. DOI: 10.1093/ajcp/84.1.24. View

Vogl T, Tenbrock K, Ludwig S, Leukert N, Ehrhardt C, van Zoelen M . Mrp8 and Mrp14 are endogenous activators of Toll-like receptor 4, promoting lethal, endotoxin-induced shock. Nat Med. 2007; 13(9):1042-9. DOI: 10.1038/nm1638. View

Zager R, Johannes G, Tuttle S, Sharma H . Acute amino acid nephrotoxicity. J Lab Clin Med. 1983; 101(1):130-40. View

Vallee B, Falchuk K . The biochemical basis of zinc physiology. Physiol Rev. 1993; 73(1):79-118. DOI: 10.1152/physrev.1993.73.1.79. View

10.

Tanner L, Nanto-Salonen K, Venetoklis J, Kotilainen S, Niinikoski H, Huoponen K . Nutrient intake in lysinuric protein intolerance. J Inherit Metab Dis. 2007; 30(5):716-21. DOI: 10.1007/s10545-007-0558-2. View

11.

Tanner L, Niinikoski H, Nanto-Salonen K, Simell O . Combined hyperlipidemia in patients with lysinuric protein intolerance. J Inherit Metab Dis. 2010; 33 Suppl 3:S145-50. DOI: 10.1007/s10545-010-9050-5. View

12.

Dirocco M, Garibotto G, Rossi G, Caruso U, Taccone A, Picco P . Role of haematological, pulmonary and renal complications in the long-term prognosis of patients with lysinuric protein intolerance. Eur J Pediatr. 1993; 152(5):437-40. DOI: 10.1007/BF01955906. View

13.

Holzinger D, Fassl S, de Jager W, Lohse P, Rohrig U, Gattorno M . Single amino acid charge switch defines clinically distinct proline-serine-threonine phosphatase-interacting protein 1 (PSTPIP1)-associated inflammatory diseases. J Allergy Clin Immunol. 2015; 136(5):1337-45. PMC: 6591125. DOI: 10.1016/j.jaci.2015.04.016. View

14.

Sampson B, Fagerhol M, Sunderkotter C, Golden B, Richmond P, Klein N . Hyperzincaemia and hypercalprotectinaemia: a new disorder of zinc metabolism. Lancet. 2002; 360(9347):1742-5. DOI: 10.1016/S0140-6736(02)11683-7. View

15.

Tanner L, Nanto-Salonen K, Niinikoski H, Jahnukainen T, Keskinen P, Saha H . Nephropathy advancing to end-stage renal disease: a novel complication of lysinuric protein intolerance. J Pediatr. 2007; 150(6):631-4, 634.e1. DOI: 10.1016/j.jpeds.2007.01.043. View

16.

Verzola D, Fama A, Villaggio B, DI Rocco M, Simonato A, DAmato E . Lysine triggers apoptosis through a NADPH oxidase-dependent mechanism in human renal tubular cells. J Inherit Metab Dis. 2012; 35(6):1011-9. DOI: 10.1007/s10545-012-9468-z. View

17.

Simell O, Perheentupa J, Rapola J, VISAKORPI J, Eskelin L . Lysinuric protein intolerance. Am J Med. 1975; 59(2):229-40. DOI: 10.1016/0002-9343(75)90358-7. View

18.

Steinbakk M, Naess-Andresen C, Lingaas E, Dale I, Brandtzaeg P, Fagerhol M . Antimicrobial actions of calcium binding leucocyte L1 protein, calprotectin. Lancet. 1990; 336(8718):763-5. DOI: 10.1016/0140-6736(90)93237-j. View

19.

Parto K, Kallajoki M, Aho H, Simell O . Pulmonary alveolar proteinosis and glomerulonephritis in lysinuric protein intolerance: case reports and autopsy findings of four pediatric patients. Hum Pathol. 1994; 25(4):400-7. DOI: 10.1016/0046-8177(94)90150-3. View

20.

Roseth A, Fagerhol M, Aadland E, Schjonsby H . Assessment of the neutrophil dominating protein calprotectin in feces. A methodologic study. Scand J Gastroenterol. 1992; 27(9):793-8. DOI: 10.3109/00365529209011186. View