title: Mice deficient in the ALS2 gene exhibit lymphopenia and abnormal hematopietic function.
abstract: 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.
authors.affiliation: Hematology Branch, National Heart, Lung, and Blood Institute, Bethesda, Maryland 20892-1202, USA.
authors.forename: Elizabeth A
authors.initials: EA
authors.lastname: Erie
authors.forename: Hoon
authors.initials: H
authors.lastname: Shim
authors.forename: Aleah L
authors.initials: AL
authors.lastname: Smith
authors.forename: Xian
authors.initials: X
authors.lastname: Lin
authors.forename: Keyvan
authors.initials: K
authors.lastname: Keyvanfar
authors.forename: Chengsong
authors.initials: C
authors.lastname: Xie
authors.forename: Jichun
authors.initials: J
authors.lastname: Chen
authors.forename: Huaibin
authors.initials: H
authors.lastname: Cai
mesh_terms: 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
chemical_list: C512358:Als2 protein, mouse; D020662:Guanine Nucleotide Exchange Factors