Gene:
alkylglycerone phosphate synthase
Gene ID:
192644
Symbol:
AGPS
Species:
1000
Description:
Alkylglycerone phosphate synthase (AGPS) belongs to a group of peroxisomal enzymes that catalyze the first step in ether phospholipid biosynthesis which involves the acylation of dihydroxyacetonephosphate followed by the introduction of the ether linkage by alkyl-DHAP synthase. Human AGPS shares a 92% nucleotide and amino acid sequence similarity with the guinea pig sequences, and the human sequence was identified by virtue of this homology. Both proteins contain indentical peroxisomal targeting signal 2 sequences in their N-terminal presequences. Deficiency of AGPS in peroxisomes has been found in patients with the following peroxisomal disorders: Zellweger syndrome, rhizomelic chondrodysplasia punctate and adrenoleukodystrophy.