Mice deficient in the ALS2 gene exhibit lymphopenia and abnormal hematopietic function.
One form of juvenile onset autosomal recessive amyotrophic lateral sclerosis (ALS2) has been linked to the dysfunction of the ALS2 gene. The ALS2 gene is expressed in lymphoblasts, however, whether ALS2-deficiency affects periphery blood is unclear. Here we report that ALS2 knockout (ALS2(-/-)) mice developed peripheral lymphopenia but had higher proportions of hematopoietic stem and progenitor cells in which the stem cell factor-induced cell proliferation was up-regulated. Our findings reveal a novel function of the ALS2 gene in the lymphopoiesis and hematopoiesis, suggesting that the immune system is involved in the pathogenesis of ALS2.
Erie
Elizabeth A
EA
Hematology Branch, National Heart, Lung, and Blood Institute, Bethesda, Maryland 20892-1202, USA.
Shim
Hoon
H
Smith
Aleah L
AL
Lin
Xian
X
Keyvanfar
Keyvan
K
Xie
Chengsong
C
Chen
Jichun
J
Cai
Huaibin
H
D000818:Animals; D001854:Bone Marrow Cells / Q000378:metabolism / Q000473:pathology; D019070:Cell Lineage; D049109:Cell Proliferation; D016922:Cellular Senescence; D020662:Guanine Nucleotide Exchange Factors / Q000172:deficiency* / Q000235:genetics* / Q000378:metabolism; D006410:Hematopoiesis / Q000235:genetics*; D006412:Hematopoietic Stem Cells / Q000378:metabolism / Q000473:pathology; D007958:Leukocyte Count; D008231:Lymphopenia / Q000209:etiology* / Q000473:pathology; D038041:Lymphopoiesis / Q000235:genetics*; D051379:Mice; D018345:Mice, Knockout; D015854:Up-Regulation
C512358:Als2 protein, mouse; D020662:Guanine Nucleotide Exchange Factors