RGD Reference Report - Absence of mitochondrial superoxide dismutase results in a murine hemolytic anemia responsive to therapy with a catalytic antioxidant. - Rat Genome Database

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Absence of mitochondrial superoxide dismutase results in a murine hemolytic anemia responsive to therapy with a catalytic antioxidant.

Authors: Friedman, JS  Rebel, VI  Derby, R  Bell, K  Huang, TT  Kuypers, FA  Epstein, CJ  Burakoff, SJ 
Citation: Friedman JS, etal., J Exp Med. 2001 Apr 16;193(8):925-34.
RGD ID: 11035277
Pubmed: PMID:11304553   (View Abstract at PubMed)
PMCID: PMC2193409   (View Article at PubMed Central)

Manganese superoxide dismutase 2 (SOD2) is a critical component of the mitochondrial pathway for detoxification of O2(-), and targeted disruption of this locus leads to embryonic or neonatal lethality in mice. To follow the effects of SOD2 deficiency in cells over a longer time course, we created hematopoietic chimeras in which all blood cells are derived from fetal liver stem cells of Sod2 knockout, heterozygous, or wild-type littermates. Stem cells of each genotype efficiently rescued hematopoiesis and allowed long-term survival of lethally irradiated host animals. Peripheral blood analysis of leukocyte populations revealed no differences in reconstitution kinetics of T cells, B cells, or myeloid cells when comparing Sod2(+/+), Sod2(-/-), and Sod2(+/-) fetal liver recipients. However, animals receiving Sod2(-/-) cells were persistently anemic, with findings suggestive of a hemolytic process. Loss of SOD2 in erythroid progenitor cells results in enhanced protein oxidative damage, altered membrane deformation, and reduced survival of red cells. Treatment of anemic animals with Euk-8, a catalytic antioxidant with both SOD and catalase activities, significantly corrected this oxidative stress-induced condition. Such therapy may prove useful in treatment of human disorders such as sideroblastic anemia, which SOD2 deficiency most closely resembles.

RGD Manual Disease Annotations    Click to see Annotation Detail View
TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
hemolytic anemia  ISOSod2 (Mus musculus)11035277; 11035277 RGD 
hemolytic anemia  IMP 11035277 RGD 

Objects Annotated

Genes (Rattus norvegicus)
Sod2  (superoxide dismutase 2)

Genes (Mus musculus)
Sod2  (superoxide dismutase 2, mitochondrial)

Genes (Homo sapiens)
SOD2  (superoxide dismutase 2)


Additional Information