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Aminoadipate-semialdehyde dehydrogenase-phosphopantetheinyl transferase
Protein AASDHPPT PDB 2byd.png
PDB rendering based on 2byd.
Available structures
PDB Ortholog search: PDBe, RCSB
External IDs OMIM607756 MGI1914868 HomoloGene9130 GeneCards: AASDHPPT Gene
EC number 2.7.8.-
RNA expression pattern
PBB GE AASDHPPT 202170 s at tn.png
More reference expression data
Species Human Mouse
Entrez 60496 67618
Ensembl ENSG00000149313 ENSMUSG00000025894
UniProt Q9NRN7 Q9CQF6
RefSeq (mRNA) NM_015423 NM_026276
RefSeq (protein) NP_056238 NP_080552
Location (UCSC) Chr 11:
105.95 – 105.97 Mb
Chr 9:
4.29 – 4.31 Mb
PubMed search [1] [2]

L-aminoadipate-semialdehyde dehydrogenase-phosphopantetheinyl transferase is an enzyme that in humans is encoded by the AASDHPPT gene.[1][2][3]

The protein encoded by this gene is similar to Saccharomyces cerevisiae LYS5, which is required for the activation of the alpha-aminoadipate dehydrogenase in the biosynthetic pathway of lysine. Yeast alpha-aminoadipate dehydrogenase converts alpha-biosynthetic-aminoadipate semialdehyde to alpha-aminoadipate. It has been suggested that defects in the human gene result in pipecolic acidemia.[3]


  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. PMID 12815048. 
  2. ^ 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. 
  3. ^ a b "Entrez Gene: AASDHPPT aminoadipate-semialdehyde dehydrogenase-phosphopantetheinyl transferase". 

Further reading[edit]

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