Acetyl-coenzyme A transporter 1 (SLC33A1)

Acetyl-coenzyme A transporter 1 (SLC33A1, AT-1) is an endoplasmic reticulum membrane transporter responsible for importing acetyl-CoA from the cytosol into the ER lumen. This transport is critical for ER protein acetylation, which regulates the folding and maturation of secreted and membrane proteins and modulates ER-specific autophagy. SLC33A1 is essential for cellular homeostasis; its dysfunction or mutation leads to a range of neurodevelopmental and neurodegenerative disorders, including spastic paraplegia type 42, amyotrophic lateral sclerosis, and potentially autism and cancer susceptibility. The SLC33A1 protein is classified as a member of the major facilitator superfamily (MFS) and solute carrier family. No clinically approved drugs are known to specifically target this transporter, but ATP, ADP, and dATP can partially inhibit its transport function in experimental systems. The S113R mutation in SLC33A1 is associated with hereditary spastic paraplegia (SPG42) and is a potential disease biomarker. Altered expression or activity may result in critical cellular dysfunction and pathology.

Other names

SLC33A1AT-1AT1ACATNCCHLNDSPG42acetyl-CoA transporter 1solute carrier family 33 member 1

For ATP, dATP, ADP — competitive inhibition of acetyl-CoA transport by competing for the binding site; no clinically validated drug mechanism of action.