Safety and efficacy of mitapivat, an oral pyruvate kinase activator, in adults with non-transfusion dependent α-thalassaemia or β-thalassaemia: an open-label …

KHM Kuo, DM Layton, A Lal, H Al-Samkari, J Bhatia… - The Lancet, 2022 - thelancet.com
Background Patients with non-transfusion-dependent thalassaemia (NTDT), although they
do not require regular blood transfusions for survival, can still accrue a heavy burden of …

The pyruvate kinase activator mitapivat reduces hemolysis and improves anemia in a β-thalassemia mouse model

A Matte, E Federti, C Kung, PA Kosinski… - The Journal of …, 2021 - Am Soc Clin Investig
Anemia in β-thalassemia is related to ineffective erythropoiesis and reduced red cell
survival. Excess free heme and accumulation of unpaired α-globin chains impose …

[HTML][HTML] Erythropoiesis in lower-risk myelodysplastic syndromes and beta-thalassemia

MD Cappellini, AT Taher, A Verma, F Shah, O Hermine - Blood Reviews, 2023 - Elsevier
The hematologic disorders myelodysplastic syndromes and beta-thalassemia are
characterized by ineffective erythropoiesis and anemia, often managed with regular blood …

A fit-for-purpose LC–MS/MS method for the simultaneous quantitation of ATP and 2, 3-DPG in human K2EDTA whole blood

H Kim, P Kosinski, C Kung, L Dang, Y Chen… - … of Chromatography B, 2017 - Elsevier
Many hemolytic anemias results in major metabolic abnormalities: two common metabolite
abnormalities include increased levels of 2, 3-diphosphoglycerate (2, 3-DPG) and …

Pyruvate kinase activators: targeting red cell metabolism in thalassemia

KHM Kuo - Hematology, 2023 - ashpublications.org
Thalassemia is an inherited red blood cell disorder whereby the qualitative and/or
quantitative imbalance in α-to β-globin ratio results in hemolysis and ineffective …

Myostatin Knockout Limits Exercise-Induced Reduction in Bovine Erythrocyte Oxidative Stress by Enhancing the Efficiency of the Pentose Phosphate Pathway

L Zhu, C Bai, X Wang, Z Wei, M Gu, X Zhou, G Su, X Liu… - Animals, 2022 - mdpi.com
Simple Summary Myostatin (MSTN) is mainly expressed in skeletal muscle and is involved
in the regulation of skeletal muscle growth and development. Loss of MSTN results in …

Mitapivat sulfate. Pyruvate kinase isozyme R activator, Treatment of pyruvate kinase deficiency, Treatment of thalassemia, Treatment of sickle cell disease

W Barcellini, JA Giannotta, N Cecchi… - Drugs of the …, 2021 - access.portico.org
Summary Mitapivat (AG-348) is an oral, allosteric activator of pyruvate kinase (PK) that
catalyzes the final step of glycolysis. The drug is active on both wild-type and mutant …

[HTML][HTML] Mitapivat (AG-348), an oral PK-R activator, in adults with non-transfusion dependent thalassemia: a phase 2, open-label, multicenter study in progress

KHM Kuo, DM Layton, K Uhlig, M Lynch, L Liu… - Blood, 2019 - Elsevier
Background: Thalassemia is a group of inherited blood disorders in which genetic mutation
(s) in the α-and/or β-globin locus lead to excess precipitation of β-or α-globin, respectively …

[引用][C] Development and validation of a LC-MS/MS method for the simultaneous detection and quantitation of adenosine-5'-triphosphate, 2, 3-bisphosphoglyceric acid …

APDH Oberacher - 2021 - Medical University of Innsbruck