期刊
HAEMATOLOGICA-THE HEMATOLOGY JOURNAL
卷 95, 期 12, 页码 2031-2037出版社
FERRATA STORTI FOUNDATION
DOI: 10.3324/haematol.2010.022129
关键词
iron metabolism; soluble hemojuvelin; ELISA; rhRGM-c; anemia; serum
类别
Background Hemojuvelin, a critical regulator of iron homeostasis, is involved in the regulation of hepcidin expression and iron homeostasis. It is expressed both as a membrane-bound form and as a soluble one. Serum hemojuvelin can be produced by secretion following furin cleavage or by proteolytic cleavage of the membrane-bound form by matriptase 2 (TMPRSS6). These forms contribute to down-regulation of hepcidin expression upon iron deficiency or hypoxia. This study describes the development and validation of the first enzyme-linked immunosorbent assay for hemojuvelin in human serum. Design and Methods This assay is based on the use of a recombinant human repulsive guidance molecule-c peptide and a polyclonal antibody against hemojuvelin able to recognize the recombinant peptide and the native soluble hemojuvelin by immunoprecipitation. Results The enzyme-linked immunosorbent assay was validated and appeared to be a robust method with intra- and inter-coefficients of variance ranging from 2.6% to 15%. The assay was able to quantify hemojuvelin levels in a control population within a range from 0.88 to 1.14 mg/L. Patients with iron-refractory iron-deficiency anemia with a mutation in the TMPRSS6 gene were found to have lower levels of circulating hemojuvelin than those in healthy patients. The enzyme-linked immunosorbent assay also showed that soluble hemojuvelin levels were significantly higher in patients with anemia of chronic disease than in control individuals. Conclusions This enzyme-linked immunosorbent assay has a good specificity and sensitivity for the quantification of soluble hemojuvelin in human serum and could be a valuable aid to understanding the physiological role of this protein.
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