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Altered elemental profiles in neuronal ceroid lipofuscinosis
E Johansson1, U Lindh, T Westermarck
1Department of Radiation Sciences, Division of Physical Biology, Uppsala, Sweden.
Journal of Trace Elements and Electrolytes in Health and Disease
|September 1, 1990
Summary
Elemental analysis of blood cells in Neuronal Ceroid Lipofuscinosis revealed increased calcium, magnesium, and iron in patients. Antioxidant treatment did not alter iron levels but unexpectedly introduced selenium into mononuclear cells.
Area of Science:
- Biochemistry
- Hematology
- Pediatric Neurology
Background:
- Neuronal Ceroid Lipofuscinosis (NCL) comprises a group of inherited neurodegenerative disorders.
- Elemental imbalances in blood cells may contribute to NCL pathogenesis and ceroid deposition.
Purpose of the Study:
- To investigate elemental profiles in thrombocytes and mononuclear cells of infantile and juvenile NCL patients.
- To assess the impact of antioxidant and vitamin therapy on elemental concentrations in NCL.
Main Methods:
- Elemental analysis of thrombocytes and mononuclear cells from NCL patients.
- Comparison of elemental concentrations with healthy subjects.
- Monitoring elemental changes following antioxidant and vitamin treatment.
Main Results:
- Thrombocytes showed elevated calcium and magnesium, with normal iron and zinc. Mononuclear cells had increased iron and decreased zinc.
- Elevated magnesium, calcium, and iron in blood cells may indicate ceroid pigmentation end products.
- Antioxidant therapy did not significantly change iron in mononuclear cells but led to unexpected selenium detection.
Conclusions:
- Elemental alterations in blood cells are characteristic of NCL.
- The observed elemental changes may reflect ceroid accumulation.
- Selenium's presence in mononuclear cells post-treatment warrants further investigation into its biological interactions in NCL.