Mice deficient in the ALS2 gene exhibit lymphopenia and abnormal hematopietic function

Elizabeth A. Erie, Hoon Shim, Aleah L. Smith, Xian Lin, Keyvan Keyvanfar, Chengsong Xie, Jichun Chen, Huaibin Cai

Research output: Contribution to journalArticlepeer-review

4 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)226-231
Number of pages6
JournalJournal of Neuroimmunology
Issue number1-2
StatePublished - Jan 2007
Externally publishedYes


  • ALS2
  • ALS2 knockout mice
  • Amyotrophic lateral sclerosis (ALS)
  • Cytokine-stimulated proliferation
  • Hematopoietic stem and progenitor cells
  • Lymphopenia

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology
  • Neurology
  • Clinical Neurology


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