# AASDHPPT

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**L-aminoadipate-semialdehyde dehydrogenase-phosphopantetheinyl transferase** (**AASDHPPT**) is an [enzyme](/source/Enzyme) that in humans is encoded by the *AASDHPPT* [gene](/source/Gene).[1][2][3] It has been suggested that defects in the human gene result in [pipecolic acidemia](/source/Pipecolic_acidemia).[3]

The AASDHPPT protein catalyzes the [post-translational modification](/source/Post-translational_modification) [phosphopantetheinylation](/source/Phosphopantetheinylation), in which a [4'-phosphopantetheine](/source/Phosphopantetheine) (4'-PP) group derived from [coenzyme A](/source/Coenzyme_A) is [covalently](/source/Covalent_bond) attached to target proteins in both [cytosol](/source/Cytosol) and [mitochondria](/source/Mitochondrion).[4] In the cytosol, these include [fatty acid synthase](/source/Fatty_acid_synthase) (FASN), [aminoadipate semialdehyde dehydrogenase](/source/Aminoadipate-semialdehyde_dehydrogenase) (AASDH), and [aldehyde dehydrogenase 1 family member L1](/source/ALDH1L1) (ALDH1L1), whereas in mitochondria the targets are the [mitochondrial acyl carrier protein](/source/Mitochondrial_acyl_carrier_protein) (mtACP), [aldehyde dehydrogenase 1 family member L2 (ALDH1L2](/source/ALDH1L2)), and [dehydrogenase/reductase 2](/source/DHRS2) (DHRS2).[4] With the exception of DHRS2, the targets of AASDHPPT contain a [acyl carrier protein](/source/Acyl_carrier_protein) (ACP) domain, a [cofactor](/source/Cofactor_(biochemistry)) domain widespread across bacteria and eukaryotes with roles in the synthesis of fatty acids, polyketides, and nonribosomal peptides.[5]

The protein encoded by this gene is similar to *[Saccharomyces cerevisiae](/source/Saccharomyces_cerevisiae)* LYS5, which is required for the activation of the [alpha-aminoadipate dehydrogenase](/source/Alpha-aminoadipate_dehydrogenase) in the [biosynthetic pathway of lysine](/source/Biosynthetic_pathway_of_lysine). Yeast alpha-aminoadipate dehydrogenase converts alpha-biosynthetic-aminoadipate semialdehyde to [alpha-aminoadipate](/source/Alpha-aminoadipate).

## References

1. 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](https://doi.org/10.1074/jbc.M305459200). [PMID 12815048](https://pubmed.ncbi.nlm.nih.gov/12815048)

1. 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](https://doi.org/10.1006/mgme.2000.3138). [PMID 11286508](https://pubmed.ncbi.nlm.nih.gov/11286508)

1. ["Entrez Gene: AASDHPPT aminoadipate-semialdehyde dehydrogenase-phosphopantetheinyl transferase"](https://www.ncbi.nlm.nih.gov/gene?Db=gene&Cmd=ShowDetailView&TermToSearch=60496)

1. Norden, Pieter R.; Wedan, Riley J.; Preston, Samuel E. J.; Canfield, Morgan; Graber, Naomi; Longenecker, Jacob Z.; Pentecost, Olivia A.; McLaughlin, Elizabeth; Hart, Madeleine L. (2024-05-10). "Mitochondrial Phosphopantetheinylation is Required for Oxidative Function". [bioRxiv 10.1101/2024.05.09.592977](https://doi.org/10.1101/10.1101/2024.05.09.592977)

1. [https://www.ebi.ac.uk/interpro/entry/InterPro/IPR036736/protein/reviewed/taxonomy/uniprot/9606/#table](https://www.ebi.ac.uk/interpro/entry/InterPro/IPR036736/protein/reviewed/taxonomy/uniprot/9606/#table)

## Further reading

- Rual JF, Venkatesan K, Hao T (2005). "Towards a proteome-scale map of the human protein-protein interaction network.". *Nature*. **437** (7062): 1173–8. [Bibcode:2005Natur.437.1173R](https://ui.adsabs.harvard.edu/abs/2005Natur.437.1173R). [doi:10.1038/nature04209](https://doi.org/10.1038/nature04209). [PMID 16189514](https://pubmed.ncbi.nlm.nih.gov/16189514). [S2CID 4427026](https://api.semanticscholar.org/CorpusID:4427026)
- Gerhard DS, Wagner L, Feingold EA (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](https://doi.org/10.1101/gr.2596504). [PMC 528928](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC528928). [PMID 15489334](https://pubmed.ncbi.nlm.nih.gov/15489334)
- Mammalian Gene Collection Program Team; Strausberg, R. L.; Feingold, E. A.; Grouse, L. H. 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](/source/Proceedings_of_the_National_Academy_of_Sciences_of_the_United_States_of_America)*. **99** (26): 16899–16903. [Bibcode:2002PNAS...9916899M](https://ui.adsabs.harvard.edu/abs/2002PNAS...9916899M). [doi:10.1073/pnas.242603899](https://doi.org/10.1073/pnas.242603899). [PMC 139241](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC139241). [PMID 12477932](https://pubmed.ncbi.nlm.nih.gov/12477932)
- Zhang QH, Ye M, Wu XY (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](https://doi.org/10.1101/gr.140200). [PMC 310934](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC310934). [PMID 11042152](https://pubmed.ncbi.nlm.nih.gov/11042152)
- Hu RM, Han ZG, Song HD (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](https://ui.adsabs.harvard.edu/abs/2000PNAS...97.9543H). [doi:10.1073/pnas.160270997](https://doi.org/10.1073/pnas.160270997). [PMC 16901](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC16901). [PMID 10931946](https://pubmed.ncbi.nlm.nih.gov/10931946)
- Lai CH, Chou CY, Ch'ang LY (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](https://doi.org/10.1101/gr.10.5.703). [PMC 310876](https://www.ncbi.nlm.nih.gov/pmc/articles/PMC310876). [PMID 10810093](https://pubmed.ncbi.nlm.nih.gov/10810093)

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Adapted from the Wikipedia article [AASDHPPT](https://en.wikipedia.org/wiki/AASDHPPT) by Wikipedia contributors ([contributor history](https://en.wikipedia.org/wiki/AASDHPPT?action=history)). Available under [Creative Commons Attribution-ShareAlike 4.0 International](https://creativecommons.org/licenses/by-sa/4.0/). Changes may have been made.