FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W4281736368> ?p ?o ?g. }

• W4281736368 endingPage "6022" @default.
• W4281736368 startingPage "6016" @default.
• W4281736368 abstract "Abstract The fetal-to-adult hemoglobin switching at about the time of birth involves a shift in expression from γ-globin to β-globin in erythroid cells. Effective re-expression of fetal γ-globin can ameliorate sickle cell anemia and β-thalassemia. Despite the physiological and clinical relevance of this switch, its posttranscriptional regulation is poorly understood. Here, we identify Pumilo 1 (PUM1), an RNA-binding protein with no previously reported functions in erythropoiesis, as a direct posttranscriptional regulator of β-globin switching. PUM1, whose expression is regulated by the erythroid master transcription factor erythroid Krüppel-like factor (EKLF/KLF1), peaks during erythroid differentiation, binds γ-globin messenger RNA (mRNA), and reduces γ-globin (HBG1) mRNA stability and translational efficiency, which culminates in reduced γ-globin protein levels. Knockdown of PUM1 leads to a robust increase in fetal hemoglobin (∼22% HbF) without affecting β-globin levels in human erythroid cells. Importantly, targeting PUM1 does not limit the progression of erythropoiesis, which provides a potentially safe and effective treatment strategy for sickle cell anemia and β-thalassemia. In support of this idea, we report elevated levels of HbF in the absence of anemia in an individual with a novel heterozygous PUM1 mutation in the RNA-binding domain (p.(His1090Profs∗16); c.3267_3270delTCAC), which suggests that PUM1-mediated posttranscriptional regulation is a critical player during human hemoglobin switching." @default.
• W4281736368 created "2022-06-13" @default.
• W4281736368 creator A5004894140 @default.
• W4281736368 creator A5006897526 @default.
• W4281736368 creator A5008073292 @default.
• W4281736368 creator A5015232451 @default.
• W4281736368 creator A5017116006 @default.
• W4281736368 creator A5019537430 @default.
• W4281736368 creator A5023037620 @default.
• W4281736368 creator A5023344939 @default.
• W4281736368 creator A5025432010 @default.
• W4281736368 creator A5029255944 @default.
• W4281736368 creator A5043065526 @default.
• W4281736368 creator A5054755882 @default.
• W4281736368 creator A5067557192 @default.
• W4281736368 creator A5074803951 @default.
• W4281736368 date "2022-11-30" @default.
• W4281736368 modified "2023-10-17" @default.
• W4281736368 title "PUM1 mediates the posttranscriptional regulation of human fetal hemoglobin" @default.
• W4281736368 cites W1604555115 @default.
• W4281736368 cites W1774620689 @default.
• W4281736368 cites W1861971834 @default.
• W4281736368 cites W1968165696 @default.
• W4281736368 cites W1968286431 @default.
• W4281736368 cites W1972240128 @default.
• W4281736368 cites W1996662512 @default.
• W4281736368 cites W2001898141 @default.
• W4281736368 cites W2011352206 @default.
• W4281736368 cites W2027401034 @default.
• W4281736368 cites W2032394536 @default.
• W4281736368 cites W2040463485 @default.
• W4281736368 cites W2051985758 @default.
• W4281736368 cites W2065637733 @default.
• W4281736368 cites W2078547860 @default.
• W4281736368 cites W2102606458 @default.
• W4281736368 cites W2128016314 @default.
• W4281736368 cites W2155119455 @default.
• W4281736368 cites W2266790363 @default.
• W4281736368 cites W2334263927 @default.
• W4281736368 cites W2339459511 @default.
• W4281736368 cites W2465447473 @default.
• W4281736368 cites W2497166756 @default.
• W4281736368 cites W2755262585 @default.
• W4281736368 cites W2765136812 @default.
• W4281736368 cites W2769524667 @default.
• W4281736368 cites W2788138562 @default.
• W4281736368 cites W2897919552 @default.
• W4281736368 cites W3013151148 @default.
• W4281736368 cites W3112131082 @default.
• W4281736368 cites W313248541 @default.
• W4281736368 cites W97024544 @default.
• W4281736368 doi "https://doi.org/10.1182/bloodadvances.2021006730" @default.
• W4281736368 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/35667093" @default.
• W4281736368 hasPublicationYear "2022" @default.
• W4281736368 type Work @default.
• W4281736368 citedByCount "2" @default.
• W4281736368 countsByYear W42817363682023 @default.
• W4281736368 crossrefType "journal-article" @default.
• W4281736368 hasAuthorship W4281736368A5004894140 @default.
• W4281736368 hasAuthorship W4281736368A5006897526 @default.
• W4281736368 hasAuthorship W4281736368A5008073292 @default.
• W4281736368 hasAuthorship W4281736368A5015232451 @default.
• W4281736368 hasAuthorship W4281736368A5017116006 @default.
• W4281736368 hasAuthorship W4281736368A5019537430 @default.
• W4281736368 hasAuthorship W4281736368A5023037620 @default.
• W4281736368 hasAuthorship W4281736368A5023344939 @default.
• W4281736368 hasAuthorship W4281736368A5025432010 @default.
• W4281736368 hasAuthorship W4281736368A5029255944 @default.
• W4281736368 hasAuthorship W4281736368A5043065526 @default.
• W4281736368 hasAuthorship W4281736368A5054755882 @default.
• W4281736368 hasAuthorship W4281736368A5067557192 @default.
• W4281736368 hasAuthorship W4281736368A5074803951 @default.
• W4281736368 hasBestOaLocation W42817363681 @default.
• W4281736368 hasConcept C104317684 @default.
• W4281736368 hasConcept C105580179 @default.
• W4281736368 hasConcept C125996951 @default.
• W4281736368 hasConcept C126322002 @default.
• W4281736368 hasConcept C172680121 @default.
• W4281736368 hasConcept C173396325 @default.
• W4281736368 hasConcept C2776317666 @default.
• W4281736368 hasConcept C2778248108 @default.
• W4281736368 hasConcept C2779234561 @default.
• W4281736368 hasConcept C2779703530 @default.
• W4281736368 hasConcept C41282012 @default.
• W4281736368 hasConcept C54355233 @default.
• W4281736368 hasConcept C71924100 @default.
• W4281736368 hasConcept C81885089 @default.
• W4281736368 hasConcept C86339819 @default.
• W4281736368 hasConcept C86803240 @default.
• W4281736368 hasConcept C95444343 @default.
• W4281736368 hasConceptScore W4281736368C104317684 @default.
• W4281736368 hasConceptScore W4281736368C105580179 @default.
• W4281736368 hasConceptScore W4281736368C125996951 @default.
• W4281736368 hasConceptScore W4281736368C126322002 @default.
• W4281736368 hasConceptScore W4281736368C172680121 @default.
• W4281736368 hasConceptScore W4281736368C173396325 @default.
• W4281736368 hasConceptScore W4281736368C2776317666 @default.
• W4281736368 hasConceptScore W4281736368C2778248108 @default.

• next