Recombinant Mouse AK2

Background

AK2 is localized in the mitochondrial intermembrane space and is primarily responsible for ATP production in hematopoietic cells. AK2 catalyzes the same ADP-to-ATP conversion as AK1 but in the mitochondrial compartment, essential for maintaining cellular energy supply. Mutations in AK2 are linked to reticular dysgenesis, a severe immunodeficiency caused by disrupted leukocyte differentiation. AK2 has critical functions in energy homeostasis during apoptosis, as ATP signaling is necessary for programmed cell death. Its role extends to regulating mitochondrial biogenesis and influencing mitochondrial DNA replication. The expression of AK2 is also vital in early embryonic development and immune system maintenance.