COASY

An enzyme involved in coenzyme A biosynthesis

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Structure of COASY enzyme

Identifiers
EC number	2.7.1.33
CAS number	9026-39-1
Alt. names
IntEnz	IntEnz view
BRENDA	BRENDA entry
ExPASy	NiceZyme view
KEGG	KEGG entry
MetaCyc	metabolic pathway

COASY (Coenzyme A synthase) is an enzyme that plays a crucial role in the biosynthesis of coenzyme A (CoA), a vital cofactor involved in numerous biochemical reactions, including the citric acid cycle, fatty acid metabolism, and the synthesis and oxidation of fatty acids.

Function

COASY is responsible for the final steps in the biosynthesis of coenzyme A from pantothenate (vitamin B5). The enzyme catalyzes the conversion of 4'-phosphopantetheine to dephospho-CoA and subsequently to CoA. This process involves two main enzymatic activities: phosphopantetheine adenylyltransferase and dephospho-CoA kinase.

Structure

COASY is a bifunctional enzyme, meaning it has two distinct catalytic activities within a single polypeptide chain. The enzyme is composed of multiple domains that facilitate its dual function. The structure of COASY allows it to efficiently catalyze the sequential reactions necessary for CoA synthesis.

Clinical Significance

Mutations in the COASY gene can lead to a rare neurodegenerative disorder known as COASY protein-associated neurodegeneration (CoPAN), which is characterized by progressive movement disorders and cognitive decline. Understanding the function and structure of COASY is crucial for developing potential therapeutic strategies for CoPAN and related conditions.

Research

Research on COASY focuses on understanding its role in cellular metabolism and its implications in human health and disease. Studies often involve biochemical assays to elucidate the enzyme's mechanism, as well as genetic studies to explore the effects of mutations.

Also see

• Coenzyme A
• Pantothenate kinase-associated neurodegeneration
• Citric acid cycle
• Fatty acid metabolism