Official websites use .gov
A
.gov website belongs to an official
government organization in the United States.
Secure .gov websites use HTTPS
A lock (
) or https:// means you've safely
connected to the .gov website. Share sensitive
information only on official, secure websites.
Surface residues in the apo (A) and liganded CPS1 (B) whose replacements cause CPS1 deficiency. Disease causing mutations in the CPS1 gene were mapped to 3-dimensional structures of the apo CPS1 (5DOT) and liganded CPS1 (5DOU) enzymes. Surface residues in the apo and liganded CPS1 are shown in magenta. CPS1 glutaminase domain is shown in light gray, synthetase domain is shown in dark gray and the linker connecting the two domains is shown in yellow.
