Hypophosphatasia

Biomineralization is a process characterized by the deposition of minerals outside or inside the cells of many organisms. Alkaline phosphatase (ALP) is represented by several enzymes that hydrolyze the esters of monophosphate (Orimo H, J Nippon Med Sch 77:1–12, 2010). Nonspecific woven alkaline phosphatase (TNSALP) hydrolyzes inorganic pyrophosphate (PPi) and makes inorganic phosphate (Pi) available for mineralization. PPi, pyridoxal 5-phosphate (PLP) (the active form of vitamin B6), and phosphoethanolamine (PEA) are the substrates of TNSALP. Hypophosphatasia (HPP) is a rare and heterogeneous metabolic disease of the bone resulting from the loss of enzyme activity resulting from mutations in the gene that encodes it [tissue-nonspecific alkaline phosphatase gene (ALPL)].