ACSBG-Acyl-CoA Synthetase Bubblegum Family | Elisa - Clia - Antibody - Protein

Background

Acyl-CoA Synthetase Bubblegum (ACSBG) family enzymes are a subset of acyl-CoA synthetases, which play crucial roles in lipid metabolism. These enzymes are responsible for catalyzing the activation of long-chain fatty acids by converting them into acyl-CoA, a key step in fatty acid metabolism. The name "Bubblegum" originates from a specific gene mutation identified in a Drosophila melanogaster mutant with a bubblegum-like phenotype.ACSBG1 play a central role in brain very long-chain fatty acids metabolism and myelinogenesis. ACSBG2 Can activate diverse saturated, monosaturated and polyunsaturated fatty acids. Has increased ability to activate oleic and linoleic acid. May play a role in spermatogenesis.

Protein Structure

The structure of ACSBG family enzymes includes several characteristic features:

• ATP Binding Domain: Essential for the binding and hydrolysis of ATP, providing the energy required for the activation of fatty acids.
• Fatty Acid Binding Site: Specific regions that bind to long-chain fatty acids, positioning them for activation.
• Catalytic Core: Contains key residues involved in the formation of acyl-CoA from fatty acids and CoA.

Classification and Subtypes

The ACSBG family is classified based on their substrate specificity and tissue distribution. Key members include:

• ACSBG1 (Bubblegum): Primarily involved in the metabolism of very long-chain fatty acids.
• ACSBG2: Similar in function to ACSBG1 but with distinct tissue-specific expression patterns.

Function and Biological Significance

ACSBG enzymes serve critical roles in various metabolic processes:

1. Fatty Acid Activation: Convert long-chain fatty acids into their respective acyl-CoA derivatives, a necessary step for subsequent metabolic processes such as β-oxidation and lipid biosynthesis.
2. Energy Production: Facilitate the breakdown of fatty acids in mitochondria and peroxisomes, contributing to cellular energy production.
3. Lipid Storage and Synthesis: Involved in the synthesis of complex lipids and storage of energy in the form of triglycerides.

Interactions

ACSBG enzymes interact with various molecules and pathways:

1. Coenzyme A (CoA): A crucial co-substrate in the formation of acyl-CoA.
2. ATP: Provides the energy required for the activation of fatty acids.
3. Lipid Metabolic Pathways: Interact with enzymes involved in fatty acid β-oxidation, lipid biosynthesis, and storage.

Clinical Significance

Dysfunction or mutations in ACSBG enzymes can lead to various metabolic disorders:

1. Inherited Metabolic Disorders: Mutations in ACSBG1 have been associated with diseases such as Adrenomyeloneuropathy (AMN) and other peroxisomal disorders, characterized by the accumulation of very long-chain fatty acids.
2. Energy Metabolism Disorders: Impaired ACSBG function can disrupt normal fatty acid metabolism, leading to energy production deficiencies and related metabolic syndromes.
3. Potential Therapeutic Targets: Given their crucial role in lipid metabolism, ACSBG enzymes are potential targets for therapeutic intervention in metabolic diseases.

Summary

The Acyl-CoA Synthetase Bubblegum (ACSBG) family includes enzymes essential for the activation of long-chain fatty acids, converting them into acyl-CoA derivatives. This activation is critical for fatty acid metabolism, including β-oxidation and lipid biosynthesis. The ACSBG family comprises key members such as ACSBG1 and ACSBG2, each with specific functions and tissue distributions. Dysfunctions or mutations in these enzymes can lead to metabolic disorders, making them important targets for understanding and potentially treating diseases related to lipid metabolism. Understanding the structure, function, interactions, and clinical implications of ACSBG enzymes provides valuable insights into their roles in health and disease.