Alkyldihydroxyacetonephosphate Synthase, Peroxisomal (AGPS) Reagents

Alkyldihydroxyacetonephosphate synthase, peroxisomal

Catalyzes the exchange of the acyl chain in acyl-dihydroxyacetonephosphate (acyl-DHAP) for a long chain fatty alcohol, yielding the first ether linked intermediate, i.e. alkyl-dihydroxyacetonephosphate (alkyl-DHAP), in the pathway of ether lipid biosynthesis.

Rhizomelic chondrodysplasia punctata 3 (RCDP3)

Peroxisome membrane. Peroxisome.

FAD-binding oxidoreductase/transferase type 4 family

AAG5; ADAP-S; ADAS; ADAS_HUMAN; ADHAPS; ADPS; Aging associated gene 5 protein; Aging-associated gene 5 protein; aging-associated protein 5; AGPS; ALDHPSY; Alkyl-DHAP synthase; Alkyldihydroxyacetonephosphate synthase; Alkyldihydroxyacetonephosphate synthase, peroxisomal; Alkylglycerone phosphate synthase; Alkylglycerone-phosphate synthase; peroxisomal; RCDP3

This gene is a member of the FAD-binding oxidoreductase/transferase type 4 family. It encodes a protein that catalyzes the second step of ether lipid biosynthesis in which acyl-dihydroxyacetonephosphate (DHAP) is converted to alkyl-DHAP by the addition of a long chain alcohol and the removal of a long-chain acid anion. The protein is localized to the inner aspect of the peroxisomal membrane and requires FAD as a cofactor. Mutations in this gene have been associated with rhizomelic chondrodysplasia punctata, type 3 and Zellweger syndrome.