| Title: | L-glutamine for sickle cell disease: more than reducing redox |
| Author(s): | Jafri F; Seong G; Jang T; Cimpeanu E; Poplawska M; Dutta D; Lim SH; |
| Address: | "Division of Hematology and Oncology, Department of Medicine, SUNY Downstate Health Sciences University, Brooklyn, NY, USA. Division of Internal Medicine, Department of Medicine, SUNY Downstate Health Sciences University, Brooklyn, NY, USA. Division of Hematology and Oncology, Department of Medicine, SUNY Upstate Medical University, 750 E Adams St, CWB Room 2284, Syracuse, NY, 13210, USA. Division of Hematology and Oncology, Department of Medicine, SUNY Upstate Medical University, 750 E Adams St, CWB Room 2284, Syracuse, NY, 13210, USA. seahhlim@yahoo.com" |
| DOI: | 10.1007/s00277-022-04867-y |
| ISSN/ISBN: | 1432-0584 (Electronic) 0939-5555 (Linking) |
| Abstract: | "Oxidative stress is a major contributor to the pathophysiology of sickle cell disease (SCD) including hemolysis and vaso-occlusive crisis (VOC). L-glutamine is a conditionally essential amino acid with important roles, including the synthesis of antioxidants, such as reduced glutathione and the cofactors NAD(H) and NADP(H), as well as nitric oxide. Given the increased levels of oxidative stress and lower (NADH):(NAD + + NADH) ratio in sickle erythrocytes that adversely affects the blood rheology compared to normal red blood cells, L-glutamine was investigated for its therapeutic potential to reduce VOC. While L-glutamine was approved by the United States (US) Food and Drug Administration to treat SCD, its impact on the redox environment in sickle erythrocytes is not fully understood. The mechanism through which L-glutamine reduces VOC in SCD is also not clear. In this paper, we will summarize the results of the Phase 3 study that led to the approval of L-glutamine for treating SCD and discuss its assumed mechanisms of action. We will examine the role of L-glutamine in health and propose how the extra-erythrocytic functions of L-glutamine might contribute to its beneficial effects in SCD. Further research into the role of L-glutamine on extra-erythrocyte functions might help the development of an improved formulation with more efficacy" |
| Keywords: | "*Anemia, Sickle Cell/drug therapy Glutamine/therapeutic use *Hemoglobinopathies/drug therapy Humans NAD/therapeutic use Oxidation-Reduction *Volatile Organic Compounds/therapeutic use Antioxidant Extra-erythrocytic functions L-glutamine Mechanisms of acti;" |
| Notes: | "MedlineJafri, Firas Seong, Gyuhee Jang, Tim Cimpeanu, Emanuela Poplawska, Maria Dutta, Dibyendu Lim, Seah H eng Review Germany 2022/05/15 Ann Hematol. 2022 Aug; 101(8):1645-1654. doi: 10.1007/s00277-022-04867-y. Epub 2022 May 14" |
