3′-Phosphoadenosine-5′-phosphosulfate (PAPS) is a derivative of adenosine monophosphate (AMP) that is phosphorylated at the 3′ position and has a sulfate group attached to the 5′ phosphate. It is the most common coenzyme in sulfotransferase reactions and hence part of sulfation pathways. It is endogenously synthesized by organisms via the phosphorylation of adenosine 5′-phosphosulfate (APS), an intermediary metabolite. In humans such reaction is performed by bifunctional 3′-phosphoadenosine 5′-phosphosulfate synthases using ATP as the phosphate donor.
In enzymology, a sulfate adenylyltransferase is an enzyme that catalyzes the chemical reaction
Methylenetetrahydrofolate dehydrogenase, cyclohydrolase and formyltetrahydrofolate synthetase 1 (MTHFD1) is a gene located in humans on chromosome 14 that encodes a protein, C-1-tetrahydrofolate synthase, cytoplasmic also known as C1-THF synthase, with three distinct enzymatic activities.
Sulfotransferase family cytosolic 2B member 1 is an enzyme that in humans is encoded by the SULT2B1 gene.
Folylpolyglutamate synthase, mitochondrial is an enzyme that in humans is encoded by the FPGS gene.
Bifunctional 3'-phosphoadenosine 5'-phosphosulfate synthetase 1 is an enzyme that in humans is encoded by the PAPSS1 gene.
Lactosylceramide alpha-2,3-sialyltransferase is an enzyme that in humans is encoded by the ST3GAL5 gene.
Hyaluronan synthase 3 is an enzyme that in humans is encoded by the HAS3 gene.
2'-5'-oligoadenylate synthetase 2 is an enzyme that in humans is encoded by the OAS2 gene.
Galactosylceramide sulfotransferase is an enzyme that in humans is encoded by the GAL3ST1 gene.
CMP-sialic acid transporter is a protein that in humans is encoded by the SLC35A1 gene.
The ATP5MC2 gene is one of three human paralogs that encode membrane subunit c of the mitochondrial ATP synthase.
Delta-1-pyrroline-5-carboxylate synthetase (P5CS) is an enzyme that in humans is encoded by the ALDH18A1 gene. This gene is a member of the aldehyde dehydrogenase family and encodes a bifunctional ATP- and NADPH-dependent mitochondrial enzyme with both gamma-glutamyl kinase and gamma-glutamyl phosphate reductase activities. The encoded protein catalyzes the reduction of glutamate to delta1-pyrroline-5-carboxylate, a critical step in the de novo biosynthesis of proline, ornithine and arginine. Mutations in this gene lead to hyperammonemia, hypoornithinemia, hypocitrullinemia, hypoargininemia and hypoprolinemia and may be associated with neurodegeneration, cataracts and connective tissue diseases. Alternatively spliced transcript variants, encoding different isoforms, have been described for this gene. As reported by Bruno Reversade and colleagues, ALDH18A1 deficiency or dominant-negative mutations in P5CS in humans causes a progeroid disease known as De Barsy Syndrome.
59 kDa 2'-5'-oligoadenylate synthetase-like protein is an enzyme that in humans is encoded by the OASL gene.
Sulfotransferase 1C4 is an enzyme that in humans is encoded by the SULT1C4 gene.
Adenosine 3'-phospho 5'-phosphosulfate transporter 1 is a protein that in humans is encoded by the SLC35B2 gene.
2'-5'-oligoadenylate synthetase 3 is an enzyme that in humans is encoded by the OAS3 gene.
CTP synthase 2 is an enzyme that in humans is encoded by the CTPS2 gene.
Tumor necrosis factor receptor superfamily member 27 is a protein that in humans is encoded by the EDA2R gene.
Spondyloepimetaphyseal dysplasia, Pakistani type is a form of spondyloepimetaphyseal dysplasia involving PAPSS2. The condition is rare.
