WikiLectures:Causes of pathological conformation of proteins
From WikiLectures
5-Aminolevulinic acid[edit | edit source]
It is formed by the condensation of glycine and succinite (succinyl-CoA) via the intermediate adipate (2-amino-3-oxo-1,6-hexanedioic acid), which is subsequently decarboxylated (removes CO2 ) to form 5-aminolevulinic acid.
The reaction is catalyzed by aminolevulate synthase (ALA-synthase, ALAS), located in the mitochondrial matrix. Pyridoxal-5-phosphate acts as a cofactor, forming a temporary Schiff base with glycine. Enzyme activity is sensitive to vitamine B6 deficiency. The product of this reaction is the starting precoursor of heme.
Odkazy[edit | edit source]
Související články[edit | edit source]
Použitá literatura[edit | edit source]
- Incomplete citation of publication. MATOUŠ, Bohuslav. Základy lekárskej chemie a biochemie. Praha : Galen, 2010. ISBN 978-80-7262-702-8.
