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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 identical 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.
Gene Name: | alkylglycerone phosphate synthase |
Synonyms: | AGPS, ALDHPSY, ADAP-S, ADPS, Alkyl-DHAP synthase, Aging-associated protein 5, ADAS, ADHAPS |
Target Sequences: | NM_003659 NP_003650.1 O00116 |
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