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ACAD-8

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Official Full Name
ACAD-8
Background
Acyl CoA dehydrogenase is the enzymeused to catalyzethe first step of β-oxidationin Fatty acid metabolism. Acyl-coenzyme A (CoA) dehydrogenases (ACADs) are a family of mitochondrial enzymes that catalyze the first dehydrogenation step in the bets-oxidation of fatty acyl-CoA derivatives. Several human ACADs exist and all ACADs catalyze the same initial dehydrogenation of the substrate at the beta-carbon atom and require electron transfer flavoprotein as an alectron acceptor. The predicted 415-amino acid ACAD8 protein contains many of the residues conserved in most other ACADs, including an active site glutamic acid residue and residues important for tetramer formation.
Synonyms
Acyl-CoA dehydrogenase family member 8 mitochondrial; ACAD-8; Isobutyryl-CoA dehydrogenase; Activator-recruited cofactor 42 kDa component; ARC42; FLJ22590
Catalog Product Name EC No. CAS No. Source Price
NATE-0801 Acyl-Coenzyme A Dehydrogenase 8 from Human, Recombinant E. coli Inquiry

Related Reading

ACAD-8, short for Acyl-CoA Dehydrogenase 8, is a critical enzyme involved in fatty acid metabolism. ACAD-8 plays an important role in fatty acid metabolism, especially in the oxidation of medium-chain fatty acids. Its structure and function make it a key player in energy homeostasis. ACAD-8 has a wide range of applications, including research and biotechnology. In addition, understanding the clinical significance of ACAD-8 highlights its importance in the diagnosis and management of metabolic disorders. Ongoing research on ACAD-8 is expected to reveal potential therapeutic interventions and future implications for the field of energy metabolism.

Structure

The functional structure of ACAD-8 includes several domains and binding sites that aid in its enzymatic activity. One of the most important domains in ACAD-8 is the catalytic domain. This domain contains the active site where the enzymatic reactions take place. Within the active site, ACAD-8 binds to fatty acyl-CoA substrates and catalyzes the dehydrogenation reaction, generating trans-enoyl-CoA intermediates. In addition to the catalytic domain, ACAD-8 often contains other domains that contribute to its overall structure and function. These additional domains may vary among different members of the Acyl-CoA Dehydrogenase family but are generally involved in substrate or cofactor binding.

Binding sites

ACAD-8 has binding sites for cofactors such as flavin adenine dinucleotide (FAD) and NAD⁺. These cofactors play key roles in the catalytic cycle of ACAD-8. FAD acts as an electron acceptor, receiving electrons in the dehydrogenation reaction, whereas NAD⁺ serves as an electron carrier, delivering electrons to the electron transport chain for further energy production.

Functions

The primary function of ACAD-8 (acyl-CoA dehydrogenase 8) is to catalyze the dehydrogenation of medium-chain fatty acyl-CoA molecules during fatty acid metabolism. This enzymatic activity is a key step in the β-oxidation pathway, a process that breaks down fatty acids to produce energy. Specifically, ACAD-8 acts on medium-chain acyl-CoA substrates that typically have carbon chain lengths between 4 and 12 carbons. As its name implies, ACAD-8 belongs to the acyl-CoA dehydrogenase family, a group of enzymes involved in fatty acid oxidation.

Enzymatic reaction

During the enzymatic reaction, ACAD-8 catalyzes the removal of two hydrogen atoms from a fatty acyl-CoA substrate to form a trans-enoyl-CoA intermediate. This dehydrogenation process transfers electrons to cofactors such as flavin adenine dinucleotide (FAD), which acts as an electron acceptor. FAD then transfers these electrons to the electron transport chain, which ultimately produces ATP (adenosine triphosphate), the cell's energy currency.

Clinical Significance

ACAD-8 has significant clinical relevance, particularly in the context of metabolic disorders. Mutations in the ACAD-8 gene have been associated with the development of fatty acid oxidation disorders, such as medium-chain acyl-CoA dehydrogenase (MCAD) deficiency. These disorders can lead to metabolic crises, including hypoglycemia, lethargy, and life-threatening complications. Early detection and management of ACAD-8-related disorders are crucial to prevent severe consequences.

Conclusion

ACAD-8 is an enzyme that catalyzes the dehydrogenation of medium-chain fatty acyl-CoA molecules during fatty acid metabolism. Its activity contributes to energy production via the β-oxidation pathway and plays a crucial role in maintaining proper cellular energy balance.