AASS - Alpha-aminoadipic semialdehyde synthase, mitochondrial - human protein (Function)
 
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AASS »  Alpha-aminoadipic semialdehyde synthase, mitochondrial
 
Protein also known as:  LKR/SDH.
Gene name:  AASS
Family names: In the N-terminal section AlaDH/PNT
In the C-terminal section Saccharopine dehydrogenase
Entry whose protein(s) existence is based on evidence at protein level
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GENE REF ISO

Function

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Overview 
Bifunctional enzyme that catalyzes the first two steps in lysine degradation. The N-terminal and the C-terminal contain lysine-ketoglutarate reductase and saccharopine dehydrogenase activity, respectively.  
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  • UniProtKB
GO molecular function 
Saccharopine dehydrogenase (NAD+, L-glutamate-forming) activitydefinition[GO:0047131]  
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  • UniProtKB
GO biological process 
L-lysine catabolic process to acetyl-CoA via saccharopinedefinition[GO:0033512]  
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  • UniPathway
Lysine catabolic processdefinition[GO:0006554]  
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  • UniProtKB
Oxidation-reduction processdefinition[GO:0055114]  
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  • UniProtKB KW
Protein tetramerizationdefinition[GO:0051262]  
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  • UniProtKB
Enzymatic activity 
This protein acts as an enzyme. It is known to catalyze the following reactions
EC 1.5.1.8: N(6)-(L-1,3-dicarboxypropyl)-L-lysine + NADP(+) + H(2)O L-lysine + 2-oxoglutarate + NADPH.  
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  • UniProtKB
EC 1.5.1.9: N(6)-(L-1,3-dicarboxypropyl)-L-lysine + NAD(+) + H(2)O L-glutamate + (S)-2-amino-6-oxohexanoate + NADH.  
1
  • UniProtKB
Pathways 
This protein is involved in the following pathways
Amino-acid degradation; L-lysine degradation via saccharopine pathway; glutaryl-CoA from L-lysine: step 1/6  
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  • UniProtKB
Amino-acid degradation; L-lysine degradation via saccharopine pathway; glutaryl-CoA from L-lysine: step 2/6  
1
  • UniProtKB
According to KEGG, this protein belongs to the following pathways:
Lysine biosynthesis  hsa00300+10157  
Lysine degradation  hsa00310+10157  
Metabolic pathways  hsa01100+10157  
According to Reactome, this protein belongs to the following pathway:
Lysine catabolism  REACT_1298  
 
More information is available from:
 

Keywords

Molecular function 
Oxidoreductase  definition   [KW-0560]
Technical term 
Multifunctional enzyme  definition   [KW-0511]
Reference proteome  definition   [KW-1185]
 

Further external links

Enzyme and pathway databases
SABIO-RK: Q9UDR5
Other
GeneWiki: AASS
GenomeRNAi: 10157
PRO: PR:Q9UDR5