Solute Carrier Family 25, Member 2 (SLC25A2)

The ORNT2 gene encodes a mitochondrial ornithine transporter that is 88% identical to that encoded by ORNT1. Northern blot analysis and PCR demonstrated highest ORNT2 expression in liver, with less significant expression in pancreas and minimal expression in kidney. Immunofluorescence analysis of transiently transfected fibroblasts from control and hyperornithinemia-hyperammonemia-homocitrullinuria syndrome (HHH) patients demonstrated that ORNT2 is localized to mitochondria.The ORNT2 cDNA is 1,412 bp, with a 186-bp 5-prime untranslated region, a 903-bp open reading frame, and a 323-bp 3-prime untranslated region. It contains a variant polyadenylation signal (CATAA) 18 bases before the terminal poly(A)