GLYATL1 is a 302 amino acid mitochondrial acyltransferase that transfers the acyl group to the N-terminus of glycine. GLYATL1 can also conjugate a multitude of substrates to form a variety of N-acylglycines. A member of the glycine N-acyltransferase family, GLYATL1 exists as two alternatively spliced isoforms and is encoded by a gene that maps to human chromosome 11q12.1. Chromosome 11 houses over 1,400 genes and comprises nearly 4% of the human genome. Jervell and Lange-Nielsen syndrome, Jacobsen syndrome, Niemann-Pick disease, hereditary angioedema and Smith-Lemli-Opitz syndrome are associated with defects in genes that maps to chromosome 11.