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Mice deficient in the ALS2 gene exhibit lymphopenia and abnormal hematopietic function
Elizabeth A Erie1, Hoon Shim, Aleah L Smith
1Hematology Branch, National Heart, Lung, and Blood Institute, Bethesda, Maryland 20892-1202, USA.
Journal of Neuroimmunology
|December 13, 2006
Summary
Amyotrophic lateral sclerosis type 2 (ALS2) gene dysfunction impacts peripheral blood. ALS2 knockout mice showed reduced lymphocytes and increased hematopoietic stem cells, revealing its role in immune cell development.
Area of Science:
- Immunology
- Genetics
- Neuroscience
Background:
- Juvenile onset autosomal recessive amyotrophic lateral sclerosis (ALS2) is linked to ALS2 gene dysfunction.
- The ALS2 gene's expression in lymphoblasts is known, but its effect on peripheral blood remains unclear.
Purpose of the Study:
- To investigate the role of ALS2 gene deficiency in peripheral blood cell populations.
- To explore the function of the ALS2 gene in lymphopoiesis and hematopoiesis.
Main Methods:
- Utilized ALS2 knockout (ALS2(-/-)) mice model.
- Analyzed peripheral blood cell counts and hematopoietic stem and progenitor cell proliferation.
Main Results:
- ALS2 knockout mice exhibited peripheral lymphopenia (reduced lymphocytes).
- A higher proportion of hematopoietic stem and progenitor cells was observed in ALS2(-/-) mice.
- Stem cell factor-induced proliferation was up-regulated in these cells.
Conclusions:
- The ALS2 gene plays a novel role in lymphopoiesis and hematopoiesis.
- Immune system involvement is suggested in the pathogenesis of ALS2-related disorders.
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