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

doi:10.1016/j.jneuroim.2006.10.019

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 journal › Article › peer-review

4 Scopus citations

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.

Original language	English (US)
Pages (from-to)	226-231
Number of pages	6
Journal	Journal of Neuroimmunology
Volume	182
Issue number	1-2
DOIs	https://doi.org/10.1016/j.jneuroim.2006.10.019
State	Published - Jan 2007
Externally published	Yes

Keywords

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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10.1016/j.jneuroim.2006.10.019

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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.",

keywords = "ALS2, ALS2 knockout mice, Amyotrophic lateral sclerosis (ALS), Cytokine-stimulated proliferation, Hematopoietic stem and progenitor cells, Lymphopenia",

author = "Erie, {Elizabeth A.} and Hoon Shim and Smith, {Aleah L.} and Xian Lin and Keyvan Keyvanfar and Chengsong Xie and Jichun Chen and Huaibin Cai",

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doi = "10.1016/j.jneuroim.2006.10.019",

language = "English (US)",

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T1 - Mice deficient in the ALS2 gene exhibit lymphopenia and abnormal hematopietic function

AU - Erie, Elizabeth A.

AU - Shim, Hoon

AU - Smith, Aleah L.

AU - Lin, Xian

AU - Keyvanfar, Keyvan

AU - Xie, Chengsong

AU - Chen, Jichun

AU - Cai, Huaibin

PY - 2007/1

Y1 - 2007/1

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AB - 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.

KW - ALS2

KW - ALS2 knockout mice

KW - Amyotrophic lateral sclerosis (ALS)

KW - Cytokine-stimulated proliferation

KW - Hematopoietic stem and progenitor cells

KW - Lymphopenia

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

Abstract

Keywords

ASJC Scopus subject areas

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