Gene/Proteome Database (LMPD)
LMPD ID
LMP002031
Gene ID
Species
Homo sapiens (Human)
Gene Name
alkylglycerone phosphate synthase
Gene Symbol
Synonyms
ADAP-S; ADAS; ADHAPS; ADPS; ALDHPSY
Alternate Names
alkyldihydroxyacetonephosphate synthase, peroxisomal; alkyl-DHAP synthase; aging-associated protein 5; aging-associated gene 5 protein; alkylglycerone-phosphate synthase
Chromosome
2
Map Location
2q31.2
EC Number
2.5.1.26
Summary
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. [provided by RefSeq, Jul 2008]
Orthologs
Proteins
| alkyldihydroxyacetonephosphate synthase, peroxisomal precursor | |
|---|---|
| Refseq ID | NP_003650 |
| Protein GI | 4501993 |
| UniProt ID | O00116 |
| mRNA ID | NM_003659 |
| Length | 658 |
| RefSeq Status | REVIEWED |
| MAEAAAAAGGTGLGAGASYGSAADRDRDPDPDRAGRRLRVLSGHLLGRPREALSTNECKARRAASAATAAPTATPAAQESGTIPKKRQEVMKWNGWGYNDSKFIFNKKGQIELTGKRYPLSGMGLPTFKEWIQNTLGVNVEHKTTSKASLNPSDTPPSVVNEDFLHDLKETNISYSQEADDRVFRAHGHCLHEIFLLREGMFERIPDIVLWPTCHDDVVKIVNLACKYNLCIIPIGGGTSVSYGLMCPADETRTIISLDTSQMNRILWVDENNLTAHVEAGITGQELERQLKESGYCTGHEPDSLEFSTVGGWVSTRASGMKKNIYGNIEDLVVHIKMVTPRGIIEKSCQGPRMSTGPDIHHFIMGSEGTLGVITEATIKIRPVPEYQKYGSVAFPNFEQGVACLREIAKQRCAPASIRLMDNKQFQFGHALKPQVSSIFTSFLDGLKKFYITKFKGFDPNQLSVATLLFEGDREKVLQHEKQVYDIAAKFGGLAAGEDNGQRGYLLTYVIAYIRDLALEYYVLGESFETSAPWDRVVDLCRNVKERITRECKEKGVQFAPFSTCRVTQTYDAGACIYFYFAFNYRGISDPLTVFEQTEAAAREEILANGGSLSHHHGVGKLRKQWLKESISDVGFGMLKSVKEYVDPNNIFGNRNLL | |
| sig_peptide: 1..58 note: includes a peroxisomal targeting signal 2 (PTS2) sequence calculated_mol_wt: 5865 peptide sequence: MAEAAAAAGGTGLGAGASYGSAADRDRDPDPDRAGRRLRVLSGHLLGRPREALSTNEC mat_peptide: 59..658 product: alkyldihydroxyacetonephosphate synthase, peroxisomal calculated_mol_wt: 67065 peptide sequence: KARRAASAATAAPTATPAAQESGTIPKKRQEVMKWNGWGYNDSKFIFNKKGQIELTGKRYPLSGMGLPTFKEWIQNTLGVNVEHKTTSKASLNPSDTPPSVVNEDFLHDLKETNISYSQEADDRVFRAHGHCLHEIFLLREGMFERIPDIVLWPTCHDDVVKIVNLACKYNLCIIPIGGGTSVSYGLMCPADETRTIISLDTSQMNRILWVDENNLTAHVEAGITGQELERQLKESGYCTGHEPDSLEFSTVGGWVSTRASGMKKNIYGNIEDLVVHIKMVTPRGIIEKSCQGPRMSTGPDIHHFIMGSEGTLGVITEATIKIRPVPEYQKYGSVAFPNFEQGVACLREIAKQRCAPASIRLMDNKQFQFGHALKPQVSSIFTSFLDGLKKFYITKFKGFDPNQLSVATLLFEGDREKVLQHEKQVYDIAAKFGGLAAGEDNGQRGYLLTYVIAYIRDLALEYYVLGESFETSAPWDRVVDLCRNVKERITRECKEKGVQFAPFSTCRVTQTYDAGACIYFYFAFNYRGISDPLTVFEQTEAAAREEILANGGSLSHHHGVGKLRKQWLKESISDVGFGMLKSVKEYVDPNNIFGNRNLL | |
Gene Information
Entrez Gene ID
Gene Name
alkylglycerone phosphate synthase
Gene Symbol
Species
Homo sapiens
Gene Ontology (GO Annotations)
| GO ID | Source | Type | Description |
|---|---|---|---|
| GO:0043231 | IDA:HPA | C | intracellular membrane-bounded organelle |
| GO:0016020 | IDA:UniProtKB | C | membrane |
| GO:0005739 | IDA:UniProt | C | mitochondrion |
| GO:0005730 | IDA:HPA | C | nucleolus |
| GO:0005782 | TAS:Reactome | C | peroxisomal matrix |
| GO:0005778 | IDA:UniProtKB | C | peroxisomal membrane |
| GO:0005777 | IDA:UniProtKB | C | peroxisome |
| GO:0071949 | ISS:UniProtKB | F | FAD binding |
| GO:0008762 | IEA:InterPro | F | UDP-N-acetylmuramate dehydrogenase activity |
| GO:0008609 | IDA:UniProtKB | F | alkylglycerone-phosphate synthase activity |
| GO:0044255 | TAS:Reactome | P | cellular lipid metabolic process |
| GO:0008611 | ISS:UniProtKB | P | ether lipid biosynthetic process |
| GO:0008610 | IDA:MGI | P | lipid biosynthetic process |
| GO:0044281 | TAS:Reactome | P | small molecule metabolic process |
KEGG Pathway Links
REACTOME Pathway Links
| REACTOME Pathway ID | Description |
|---|---|
| REACT_1407 | Plasmalogen biosynthesis |
Domain Information
InterPro Annotations
| Accession | Description |
|---|---|
| IPR025650 | Alkyldihydroxyacetonephosphate synthase |
| IPR016169 | CO dehydrogenase flavoprotein-like, FAD-binding, subdomain 2 |
| IPR006094 | FAD linked oxidase, N-terminal |
| IPR016166 | FAD-binding, type 2 |
| IPR016167 | FAD-binding, type 2, subdomain 1 |
| IPR004113 | FAD-linked oxidase, C-terminal |
| IPR016164 | FAD-linked oxidase-like, C-terminal |
UniProt Annotations
Entry Information
Gene Name
alkylglycerone phosphate synthase
Protein Entry
ADAS_HUMAN
UniProt ID
Species
Human
Comments
| Comment Type | Description |
|---|---|
| Catalytic Activity | 1-acyl-glycerone 3-phosphate + a long-chain alcohol = an alkyl-glycerone 3-phosphate + a long-chain acid anion. |
| Cofactor | Name=FAD; Xref=ChEBI |
| Disease | Rhizomelic chondrodysplasia punctata 3 (RCDP3) [MIM |
| Function | 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. |
| Pathway | Glycerolipid metabolism; ether lipid biosynthesis. |
| Similarity | Belongs to the FAD-binding oxidoreductase/transferase type 4 family. |
| Similarity | Contains 1 FAD-binding PCMH-type domain. |
| Subcellular Location | Peroxisome membrane. Note=Localized to the inner aspect of the peroxisomal membrane. |
| Subunit | Homodimer. |
Identical and Related Proteins
Unique RefSeq proteins for LMP002031 (as displayed in Record Overview)
| Protein GI | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| 4501993 | RefSeq | NP_003650 | 658 | alkyldihydroxyacetonephosphate synthase, peroxisomal precursor |
Identical Sequences to LMP002031 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:4501993 | DBBJ | BAG36924.1 | 658 | unnamed protein product [Homo sapiens] |
| GI:4501993 | GenBank | AAI41821.1 | 658 | Alkylglycerone phosphate synthase [Homo sapiens] |
| GI:4501993 | GenBank | ACP87521.1 | 658 | Sequence 163 from patent US 7504211 |
| GI:4501993 | GenBank | ACS08198.1 | 658 | Sequence 163 from patent US 7537891 |
| GI:4501993 | GenBank | ADA18743.1 | 658 | Sequence 567 from patent US 7608413 |
| GI:4501993 | GenBank | AEN34639.1 | 658 | Sequence 567 from patent US 7998689 |
Related Sequences to LMP002031 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:4501993 | GenBank | EAX11058.1 | 684 | alkylglycerone phosphate synthase, isoform CRA_b [Homo sapiens] |
| GI:4501993 | GenBank | ADA18744.1 | 684 | Sequence 568 from patent US 7608413 |
| GI:4501993 | GenBank | AEN34640.1 | 684 | Sequence 568 from patent US 7998689 |
| GI:4501993 | GenBank | JAA19929.1 | 658 | alkylglycerone phosphate synthase [Pan troglodytes] |
| GI:4501993 | GenBank | JAA44733.1 | 658 | alkylglycerone phosphate synthase [Pan troglodytes] |
| GI:4501993 | RefSeq | XP_001154263.4 | 684 | PREDICTED: alkyldihydroxyacetonephosphate synthase, peroxisomal isoform X1 [Pan troglodytes] |