Dihydrodipicolinate Synthase Like Protein (DHDPSL)

By searching for genes in a region of chromosome 10 linked to primary hyperoxalurea type III, followed by PCR of genomic DNA, Belostotsky et al. (2010) cloned DHDPSL. They noted that, despite the designation 'mitochondrial dihydrodipicolinate synthase-like,' DHDPSL shares little homology with E. coli dihydrodipicolinate synthase (Dhdps), particularly in the putative substrate-binding region. Moreover, neither lysine biosynthesis nor sialic acid metabolism, for which Dhdps is responsible, occurs in vertebrate mitochondria. Instead, Belostotsky et al. (2010) speculated that DHDPSL encodes mitochondrial 4-hydroxyl-2-oxoglutarate aldolase (EC 4.1.3.16), which catalyzes the final step in the metabolic pathway of hydroxyproline, releasing the products glyoxylate and pyruvate. DHDPSL is predominantly expressed in liver and kidney.