OAZ2
Description
The OAZ2 (ornithine decarboxylase antizyme 2) is a protein-coding gene located on chromosome 15.
OAZ2, encoded by the OAZ2 gene, is an enzyme involved in the regulation of polyamine synthesis. Polyamines are essential for cell growth and development, and their levels are tightly controlled. OAZ2 acts as an antizyme, binding to and inhibiting ornithine decarboxylase (ODC), the enzyme responsible for the first step in polyamine biosynthesis. OAZ2 expression is regulated by polyamines, with increased levels leading to enhanced OAZ2 production. Unlike other antizymes, OAZ2 does not accelerate the degradation of ODC, but rather inhibits its activity.
OAZ2 is an ODC antizyme protein that negatively regulates ODC activity, intracellular polyamine biosynthesis, and uptake in response to increased intracellular polyamine levels. It binds to ODC monomers, inhibiting the assembly of functional ODC homodimers. OAZ2 does not target the ODC monomers for degradation, allowing for a protein synthesis-independent restoration of ODC activity. OAZ2 is involved in the translocation of AZIN2 from the ER-Golgi intermediate compartment (ERGIC) to the cytosol. It interacts with ODC1 and sterically blocks ODC homodimerization. OAZ2 also interacts with AZIN2, disrupting the interaction between the antizyme and ODC1.
OAZ2 is also known as AZ2.
Associated Diseases
- microcytic anemia with liver iron overload
- hemoglobin E-beta-thalassemia syndrome
- hemochromatosis type 5
- dehydrated hereditary stomatocytosis
- hemoglobin D disease
- hypoparathyroidism, familial isolated, 2
- familial pseudohyperkalemia
