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Literature summary for 1.2.1.31 extracted from

  • Struys, E.A.; Jakobs, C.
    Metabolism of lysine in alpha-aminoadipic semialdehyde dehydrogenase-deficient fibroblasts: evidence for an alternative pathway of pipecolic acid formation (2010), FEBS Lett., 584, 181-186.
    View publication on PubMed
Search for more literature for 1.2.1.31

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
fibroblast
-
 Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
L-alpha-aminoadipate-semialdehyde + NAD(P)+ + H2O
-
 Homo sapiens L-alpha-aminoadipate + NAD(P)H + H+
-
 ?

Synonyms

Synonyms Comment Organism
alpha-AASA dehydrogenase
-
 Homo sapiens
alpha-aminoadipic semialdehyde dehydrogenase
-
 Homo sapiens
antiquitin
-
 Homo sapiens

Cofactor

Cofactor Comment Organism Structure
NAD(P)+
-
 Homo sapiens

General Information

General Information Comment Organism
malfunction alpha-AASA dehydrogenase deficiency results in the accumulation of pathognomonic alpha-aminoadipic semialdehyde (in cerebrospinal fluid, plasma and urine) and pipecolic acid (cerebrospinal fluid and plasma) in affected patients Homo sapiens