Delta aminolevulinate synthase (δ-ALA synthase) is an enzyme involved in the production of heme, a crucial component of hemoglobin. The word "synthase" is pronounced /ˈsɪnθeɪz/, with the letter "y" representing the consonant sound /θ/. "Delta" is pronounced /ˈdɛltə/, with the letter "a" representing the schwa sound /ə/. "Aminolevulinate" is pronounced /əmiːnoʊlɛvjuːlɪneɪt/, with the stress on the third syllable. The word "synthase" is derived from the Greek word "synthesis", meaning "putting together". Delta aminolevulinate synthase is an important enzyme in the production of hemoglobin and other heme-containing proteins.
Delta aminolevulinate synthase (also known as ALAS - aminolevulinate synthase) is an enzyme involved in the biosynthesis of heme, an essential component of hemoglobin and various other proteins in the body. It catalyzes the conversion of glycine and succinyl-CoA to delta-aminolevulinate (ALA), a key precursor in the production of heme in the mitochondria.
ALAS is the first and rate-limiting enzyme in the heme biosynthesis pathway. The reaction, facilitated by this enzyme, takes place in the mitochondrial matrix. The enzyme binds succinyl-CoA and glycine, forming a Schiff base intermediate. This intermediate undergoes a series of transformations, leading to the production of ALA, which is released from the enzyme's active site.
Delta aminolevulinate synthase is crucial for maintaining normal heme levels in the body. Dysregulation or deficiency of this enzyme can lead to various disorders, such as X-linked sideroblastic anemia and erythropoietic protoporphyria, which are characterized by impaired heme production.
The activity of delta aminolevulinate synthase is regulated by several factors, including cellular heme levels and iron availability. Feedback inhibition by heme and environmental factors, such as drug exposure, can modulate the expression and activity of this enzyme.
Research on delta aminolevulinate synthase aims to understand its molecular structure, cellular regulation, and potential therapeutic targets for diseases related to heme synthesis.