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Cited by 122S100 Proteins and AnnexinsTrace Elements in HealthIron Metabolism and Disorders

The DOI matches its Crossref record; 33 of the 33 references it cites were found in a registry or an index.

Hyperzincaemia and hypercalprotectinaemia: a new disorder of zinc metabolism

Sampson, Barry, Fagerhol, Magne K, Sunderkötter, Cord, Golden, Barbara E, Richmond, Peter, Klein, Nigel, Kovar, Ilya Z, Beattie, John H, Wolska-Kusnierz, Beata, Saito, Yoshiaki, Roth, Johannes·2002·The Lancet
DOI 10.1016/s0140-6736(02)11683-7
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Abstract

Background Calprotectin (complex of S100A8 and S100A9) is the major calcium and zinc-binding protein of phagocytes. We report a new syndrome with recurrent infections, inflammation, and hyperzincaemia associated with excessively high plasma concentrations of calprotectin.Methods We measured calprotectin in plasma and protein fractions by ELISA assay and zinc by atomic absorption spectrometry. Plasma proteins were fractionated by size exclusion chromatography and electrophoresis. Mass spectra of purified proteins were determined by MALDI-TOFMS.Findings We assessed five patients, two of whom are related. All patients had much the same biochemical findings of hyperzincaemia (77-200 micromol/L, reference range 11-18 micromol/L) and raised plasma calprotectin concentrations (1.4-6.5 g/L, reference range <1 mg/L). All patients presented with recurrent infections, hepatosplenomegaly, anaemia, and evidence of systemic inflammation. Three patients had cutaneous inflammation and three presented in infancy with severe growth failure. Size exclusion chromatography showed that zinc and calprotectin were associated in a broad fraction with molecular weight range 100-300 kDa. Analysis by electrophoresis and mass spectrometry showed that the patients' protein contained normal S100A8 and S100A9 subunits.Interpretation Dysregulation of zinc metabolism associated with accumulation in plasma of S100A8 and S100A9 defines a new disease, which encompasses a pathological role for dysregulation of two members of the large S100 protein family.

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Crossref · 10.1016/s0140-6736(02)11683-7

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  1. Richmond, 1999, Dysregulation of calprotectin metabolism is associated with a multisystem autoimmune vasculitis, Arch Dis Child, 80, 67A

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Metadata

Authors
Sampson, B., Fagerhol, M. K., Sunderkötter, C., Golden, B. E., Richmond, P., Klein, N., Kovar, I. Z., Beattie, J. H., Wolska-Kusnierz, B., Saito, Y., & Roth, J.
Affiliations
  • Charing Cross Hospital (GB)
  • Oslo University Hospital (NO)
  • University of Münster (DE)
  • University of Aberdeen (GB)
  • Chelsea and Westminster Hospital (GB)
  • Tokyo Women's Medical University (JP)
Corresponding author
Sampson, Barry
ORCID
Venue
The Lancet
Publisher
Elsevier BV
Published
2002-11
Volume
360
Issue
9347
Pages
1742-1745
ISSN
  • 0099-5355 (ISSN-L)
  • 0140-6736
DOI
10.1016/s0140-6736(02)11683-7
OpenAlex
W2145976476
Cited by
122
References
33
Citations by year
2026: 1 · 2025: 6 · 2024: 1 · 2023: 2 · 2022: 4 · 2021: 4 · 2020: 1 · 2019: 4 · 2018: 4 · 2017: 2 · 2016: 7 · 2015: 5 · 2014: 4 · 2013: 6 · 2012: 12
FWCI
2.20
Type
journal article
Language
en
Open access
closed
Licence
www.elsevier.com/tdm/userlicense/1.0/ (tdm)
Locations
  • The Lancet (publishedVersion)
  • PubMed (publishedVersion)
  • UWA Profiles and Research Repository (UWA) (submittedVersion)
  • UCL Discovery (University College London) (submittedVersion)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › S100 Proteins and Annexins
  • Health Sciences › Nursing › Nutrition and Dietetics › Trace Elements in Health
  • Health Sciences › Medicine › Hematology › Iron Metabolism and Disorders
Keywords
calprotectin, S100A8, S100A9, recurrent infections, systemic inflammation, hepatosplenomegaly, anemia, skin inflammation, growth failure, plasma protein fractionation, S-100 protein
MeSH terms
Adolescent, Adult, Child, Female, Humans, Male, Syndrome, Zinc, Leukocyte L1 Antigen Complex

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