This strain was generated using embryonic stem cells provided by the International Knockout Mouse Consortium (KOMP). Hscb (iron-sulfur cluster co-chaperone or HS20/heat shock cognate protein 20) encodes a mitochondrial co-chaperone that partners with HSP9 (heat shock protein 9) and is involved in iron-sulfur cluster (Fe-S) biogenesis.&nbsp; Mutations in HSP9 are associated with congenital sideroblastic anemias (CSAs). Exons 2-4 of the Hscb gene are flanked by loxP sites in this conditional mutant strain. Heterozygous and homozygous mice are healthy, appear normal and are fertile. When bred to mice expressing a Cre recombinase in most tissues (B6.C-Tg(CMV-cre)1Cgn/J) no homozygous Hscb null embryos are observed by E7.5. When combined with mice expressing a pan-hematopoietic Cre recombinase (Tg(Vav1-cre)1Graf), resulting offspring are growth delayed and exhibit atrophic spleens, limited hematopoiesis, and die of bacterial pneumonia between P4 and P7. When combined with mice expressing an erythroid specific Cre recombinase (Eportm1(EGFP/cre)Uk) the resulting Hscbfl/- embryos die of anemia prior to E14.5. At E11.5 a subset of circulating nucleated erythrocytes contain coarse iron granules.

Exons 2-4 of the Hscb (iron-sulfur cluster co-chaperone) gene are flanked by loxP sites in this conditional mutant strain. These mice may be useful in studies of congenital sideroblastic anemias (CSAs).

This strain is hosted by NIH's MMRRC, which partners with JAX for the distribution of this and other strains. For additional information and to purchase, please visit the MMRRC site.