Recombinant Human Alkyldihydroxyacetonephosphate synthase, peroxisomal (AGPS)

Uniprot Id

O00116

Target Species

Human

Target Name

AGPS

Target Full Name

Alkyldihydroxyacetonephosphate synthase, peroxisomal

Target Function

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.

Target Involvement

Rhizomelic chondrodysplasia punctata 3 (RCDP3)

Target Subcellular Location

Peroxisome membrane. Peroxisome.

Target Protein Families

FAD-binding oxidoreductase/transferase type 4 family

Target Synonyms

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

Target Background

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.