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Alkyldihydroxyacetonephosphate synthase

Catalyzes the exchange of an acyl for a long-chain alkyl group and the formation of the ether bond in the biosynthesis of ether phospholipids.

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Alkyldihydroxyacetonephosphate synthase

Also known as Alkyldihydroxyacetonephosphate synthase (Alkyl-DHAP synthase) (Alkylglycerone-phosphate synthase).
Catalyzes the exchange of an acyl for a long-chain alkyl group and the formation of the ether bond in the biosynthesis of ether phospholipids.

Alkyldihydroxyacetonephosphate synthase, peroxisomal

Also known as Alkyldihydroxyacetonephosphate synthase, peroxisomal (Alkyl-DHAP synthase) (Aging-associated gene 5 protein) (Alkylglycerone-phosphate synthase).
AGPS: Defects in AGPS are the cause of rhizomelic chondrodysplasia punctata type 3 (RCDP3). RCDP3 is characterized by rhizomelic shortening of femur and humerus, vertebral disorders, cataract, cutaneous lesions and severe mental retardation. Belongs to the FAD-binding oxidoreductase/transferase type 4 family.

Protein type: EC 2.5.1.26; Lipid Metabolism - ether lipid; Mitochondrial; Oxidoreductase; Transferase

Chromosomal Location of Human Ortholog: 2q31.2

Cellular Component: intracellular membrane-bound organelle; membrane; mitochondrion; nucleolus; peroxisomal matrix; peroxisomal membrane; peroxisome

Molecular Function: alkylglycerone-phosphate synthase activity; protein binding

Biological Process: ether lipid biosynthetic process; lipid biosynthetic process

Disease: Rhizomelic Chondrodysplasia Punctata, Type 3

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