InterPro domain: IPR009395

GCN5L1 (general control of amino acid synthesis 5-like 1), also known as BLOC1S1 (biogenesis of lysosome-related organelles complex 1 subunit 1). It is a component of the BLOC-1 complex, a complex required for normal biogenesis of lysosome-related organelles [ 1 ]. GCN5L1 has been identified as a critical component of the mitochondrial protein acetylation programme [ 2 , 3 ]. It acts as a nutrient-sensing regulator that controls mitochondrial content through coordinated regulation of mitochondrial biogenesis and autophagy [ 4 ].

1. BLOC-1 is required for cargo-specific sorting from vacuolar early endosomes toward lysosome-related organelles. Mol. Biol. Cell 18, 768-80
2. Identification of a molecular component of the mitochondrial acetyltransferase programme: a novel role for GCN5L1. Biochem. J. 443, 655-61
3. Mitochondrial protein acetylation mediates nutrient sensing of mitochondrial protein synthesis and mitonuclear protein balance. IUBMB Life 66, 793-802
4. GCN5-like protein 1 (GCN5L1) controls mitochondrial content through coordinated regulation of mitochondrial biogenesis and mitophagy. J. Biol. Chem. 289, 2864-72