L-aminoadipate-semialdehyde dehydrogenase-phosphopantetheinyl transferase (AASDHPPT ) is an enzyme that in humans is encoded by the AASDHPPT gene .[ 5] [ 6] [ 7] pipecolic acidemia .[ 7]
The AASDHPPT protein catalyzes the post-translational modification phosphopantetheinylation , in which a 4'-phosphopantetheine (4'-PP) group derived from coenzyme A is covalently attached to target proteins in both cytosol and mitochondria .[ 8] fatty acid synthase (FASN), aminoadipate semialdehyde dehydrogenase (AASDH), and aldehyde dehydrogenase 1 family member L1 (ALDH1L1), whereas in mitochondria the targets are the mitochondrial acyl carrier protein (mtACP), aldehyde dehydrogenase 1 family member L2 (ALDH1L2 ), and dehydrogenase/reductase 2 (DHRS2).[ 8] acyl carrier protein (ACP) domain, a cofactor domain widespread across bacteria and eukaryotes with roles in the synthesis of fatty acids, polyketides, and nonribosomal peptides.[ 9]
The protein encoded by this gene is similar to Saccharomyces cerevisiae alpha-aminoadipate dehydrogenase in the biosynthetic pathway of lysine . Yeast alpha-aminoadipate dehydrogenase converts alpha-biosynthetic-aminoadipate semialdehyde to alpha-aminoadipate .
1 2 3 GRCh38: Ensembl release 89: ENSG00000149313 – Ensembl , May 20171 2 3 GRCm38: Ensembl release 89: ENSMUSG00000025894 – Ensembl , May 2017↑ "Human PubMed Reference:" . National Center for Biotechnology Information, U.S. National Library of Medicine .↑ "Mouse PubMed Reference:" . National Center for Biotechnology Information, U.S. National Library of Medicine .↑ Joshi AK, Zhang L, Rangan VS, Smith S (Aug 2003). "Cloning, expression, and characterization of a human 4'-phosphopantetheinyl transferase with broad substrate specificity" . J Biol Chem . 278 (35): 33142– 9. doi :10.1074/jbc.M305459200 PMID 12815048 . ↑ Praphanphoj V, Sacksteder KA, Gould SJ, Thomas GH, Geraghty MT (Apr 2001). "Identification of the alpha-aminoadipic semialdehyde dehydrogenase-phosphopantetheinyl transferase gene, the human ortholog of the yeast LYS5 gene". Mol Genet Metab . 72 (4): 336– 42. doi :10.1006/mgme.2000.3138 . PMID 11286508 . 1 2 "Entrez Gene: AASDHPPT aminoadipate-semialdehyde dehydrogenase-phosphopantetheinyl transferase" .1 2 Norden PR, Wedan RJ, Preston SE, Canfield M, Graber N, Longenecker JZ, Pentecost OA, McLaughlin E, Hart ML (2024-05-10). "Mitochondrial Phosphopantetheinylation is Required for Oxidative Function". bioRxiv 10.1101/2024.05.09.592977 ↑ https://www.ebi.ac.uk/interpro/entry/InterPro/IPR036736/protein/reviewed/taxonomy/uniprot/9606/#table
Rual JF, Venkatesan K, Hao T, et al. (2005). "Towards a proteome-scale map of the human protein-protein interaction network". Nature . 437 (7062): 1173– 8. Bibcode :2005Natur.437.1173R . doi :10.1038/nature04209 . PMID 16189514 . S2CID 4427026 . Gerhard DS, Wagner L, Feingold EA, et al. (2004). "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)" . Genome Res . 14 (10B): 2121– 7. doi :10.1101/gr.2596504 . PMC 528928 PMID 15489334 . Mammalian Gene Collection Program Team, Strausberg RL, Feingold EA, Grouse LH, et al. (2002). "Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences" . Proceedings of the National Academy of Sciences 99 (26): 16899– 16903. Bibcode :2002PNAS...9916899M . doi :10.1073/pnas.242603899 PMC 139241 PMID 12477932 . Zhang QH, Ye M, Wu XY, et al. (2001). "Cloning and functional analysis of cDNAs with open reading frames for 300 previously undefined genes expressed in CD34+ hematopoietic stem/progenitor cells" . Genome Res . 10 (10): 1546– 60. doi :10.1101/gr.140200 . PMC 310934 PMID 11042152 . Hu RM, Han ZG, Song HD, et al. (2000). "Gene expression profiling in the human hypothalamus-pituitary-adrenal axis and full-length cDNA cloning" . Proc. Natl. Acad. Sci. U.S.A . 97 (17): 9543– 8. Bibcode :2000PNAS...97.9543H . doi :10.1073/pnas.160270997 PMC 16901 PMID 10931946 . Lai CH, Chou CY, Ch'ang LY, et al. (2000). "Identification of novel human genes evolutionarily conserved in Caenorhabditis elegans by comparative proteomics" . Genome Res . 10 (5): 703– 13. doi :10.1101/gr.10.5.703 . PMC 310876 PMID 10810093 .
