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Iron and iron proteins found in the genetic disease, hereditary spherocytosis
- Source :
- Inorganica Chimica Acta. 339:481-487
- Publication Year :
- 2002
- Publisher :
- Elsevier BV, 2002.
-
Abstract
- Hereditary spherocytosis (HS) is a disorder of the red blood cell (RBC) membrane, characterized by abnormally fragile and spherical RBCs. It is the predominant cause of haemolytic anaemia in people of northern European descent. Owing to the increased rate of RBC synthesis that is characteristic of the condition, it may result in an increase in absorption of iron from the diet by the affected individual. Ultimately, pathological iron overload may develop. Fortunately, the condition responds well to surgical removal of the spleen (splenectomy). In this project, iron studies were performed on a set of spleen tissue samples obtained following routine splenectomy for HS. Cell architecture and level of deposition of haemosiderin, the distinctive form of iron found in iron-loaded tissues, were assessed. The tissue iron content was determined by AAS and ICP (mean value=2276±507 μg g-1 dry tissue, values reported for normal spleens range from 972±60 to 1345±190 μg g-1). Ion exchange chromatography was employed to separate the protein content of the tissue into four chromatographic bands, notionally transferrin, ferritin, haemprotein and haemosiderin, and these proteins quantified. Mossbauer spectroscopy identified the iron as Fe(III), predominantly in ferrihydrite (5Fe2O3·9H2O) and haem environments within the tissue. Typical spectral parameters, recorded at 300 K, were: isomer shift and quadrupole splitting of 0.36 and 0.62 mm s-1, respectively, (ferrihydrite) and 0.14 and 1.97 mm s-1 (haem). The results indicate a condition corresponding to mild iron overload.
- Subjects :
- chemistry.chemical_classification
Goethite
biology
Ion chromatography
Spleen
medicine.disease
Molecular biology
Hereditary spherocytosis
Inorganic Chemistry
Ferritin
Red blood cell
Ferrihydrite
medicine.anatomical_structure
chemistry
Biochemistry
Transferrin
visual_art
Materials Chemistry
medicine
biology.protein
visual_art.visual_art_medium
Physical and Theoretical Chemistry
Subjects
Details
- ISSN :
- 00201693
- Volume :
- 339
- Database :
- OpenAIRE
- Journal :
- Inorganica Chimica Acta
- Accession number :
- edsair.doi...........2e9a0b9e350464b8cad5a4a052282f7a