%0 Journal Article
%T Amino acid signature during sickle cell pain crisis shows significant alterations related to nitric oxide and energy metabolism.
%A Zhou Y
%A Yu X
%A Nicely A
%A Cunningham G
%A Challa C
%A McKinley K
%A Nickel R
%A Campbell A
%A Darbari D
%A Summar M
%A Majumdar S
%A Zhou Y
%A Yu X
%A Nicely A
%A Cunningham G
%A Challa C
%A McKinley K
%A Nickel R
%A Campbell A
%A Darbari D
%A Summar M
%A Majumdar S
%J Mol Genet Metab
%V 137
%N 1
%D Aug 2022 20
%M 36030599
%F 4.204
%R 10.1016/j.ymgme.2022.08.004
%X Nitric oxide depletion secondary to arginase induced arginine deficiency has been shown to be important in the pathophysiology of vaso-occlusion in sickle cell pain crisis. Our objective of this study was to perform a comprehensive amino acid evaluation during sickle cell pain crisis. In a total of 58 subjects (29 in steady-state sickle cell disease and 29 with sickle cell pain crisis), the amino acids related to nitric oxide pathway was significantly decreased during sickle cell pain crisis compared to steady-state sickle cell disease: arginine (p = 0.001), citrulline (p = 0.012), and ornithine (p = 0.03). In addition, the amino acids related to energy metabolism was significantly decreased during a pain crisis: asparagine (p < 0.001), serine (p = 0.002), histidine (p = 0.017), alanine (p = 0.004), tyrosine (p = 0.012), methionine (p = 0.007), cystine (p = 0.016), isoleucine (p = 0.016) and lysine (p = 0.006). The amino acid related to oxidative stress were significantly higher during a sickle cell pain crisis (glutamic acid (p < 0.001). Furthermore, multivariate analysis with partial least squares-discriminant analysis (PLS-DA) showed that deficiencies of the amino acids arginine, asparagine, citrulline, methionine and alanine were the most important related to sickle cell pain crisis.