基因名：
PAPSS2
产品别名：
ATPSK2； BCYM4； SK2； PAPSS2； 3'-phosphoadenosine 5'-phosphosulfate synthase 2； 3'-phosphoadenosine 5'-phosphosulfate synthase 2； bifunctional 3'-phosphoadenosine 5'-phosphosulfate synthase 2； 3-prime-phosphoadenosine 5-prime-phosphosulfate synthase 2； ATP sulfurylase/APS kinase 2； ATP sulfurylase/adenosine 5'-phosphosulfate kinase； PAPS synthase 2； PAPS synthetase 2； SK 2； adenosine 5'-phosphosulfate kinase； adenylyl-sulfate kinase； bifunctional 3'-phosphoadenosine 5'-phosphosulfate synthethase 2； epididymis secretory sperm binding protein； sulfate adenylyltransferase； 腺苷酸硫酸激酶1； 腺苷酸硫酸激酶2；
背景信息：
PAPSS2 is one of the two PAPS synthetases. Three-prime-phosphoadenosine 5-prime-phosphosulfate (PAPS) is the sulfate donor cosubstrate for all sulfotransferase (SULT) enzymes. SULTs catalyze the sulfate conjugation of many endogenous and exogenous compounds, including drugs and other xenobiotics. In humans, PAPS is synthesized from adenosine 5-prime triphosphate (ATP) and inorganic sulfate by 2 isoforms, PAPSS1 and PAPSS2.
Bifunctional 3'-phosphoadenosine 5'-phosphosulfate synthetases (PAPS synthetase or PAPSS), also designated sulfurylase kinase (SK), are important for sulfate assimilation in the sulfur metabolism pathway. PAPPS proteins are bifunctional enzymes with APS kinase and ATP sulfurylase activity, which mediate two steps in the sulfate activation pathway. The PAPSS proteins belong to the APS kinase family and to the sulfate adenylyltransferase family of proteins. In mammals, PAPSS proteins are the sole source of sulfate. During postnatal growth, PAPSS proteins may play a role in skeletogenesis. Defects in the PAPSS2 gene can cause the Pakistani type of spondyloepimetaphyseal dysplasia (SEMD), an autosomal recessive form of SEMD characterized by short, bowed limbs, enlarged knee joints and mild brachydactyly.
