Notch2 Signaling Drives Cardiac Hypertrophy by Suppressing Purine Nucleotide Metabolism

Guardado en:
Detalles Bibliográficos
Publicado en:Research vol. 8 (2025)
Autor principal: Wang, Yuhong
Otros Autores: Li, Yizhe, Chen, Shihong, Yu, Tingting, Sun, Weiyan, Liu, Jiao, Ren, Huiwen, Zhou, Yao, Wang, Lu, Tao, Xixi, Du, Ronglu, Shang, Wenlong, Li, Yinxiu, Tian, Danyang, Wang, Bei, Shen, Yujun, Liu, Qian, Yu, Ying
Publicado:
Materias:
Acceso en línea:Citation/Abstract
Full Text - PDF
Etiquetas: Agregar Etiqueta
Sin Etiquetas, Sea el primero en etiquetar este registro!

MARC

LEADER 00000nab a2200000uu 4500
001 3254941535
003 UK-CbPIL
022 |a 2096-5168 
022 |a 2639-5274 
024 7 |a 10.34133/research.0635  |2 doi 
035 |a 3254941535 
045 2 |b d20250101  |b d20251231 
100 1 |a Wang, Yuhong 
245 1 |a Notch2 Signaling Drives Cardiac Hypertrophy by Suppressing Purine Nucleotide Metabolism 
260 |c 2025 
513 |a Journal Article 
520 3 |a Gain-of-function mutations of Notch2 cause the rare autosomal dominant disorder known as Hajdu–Cheney syndrome (HCS). Most patients with HCS develop congenital heart disease; however, the precise mechanisms remain elusive. Here, a murine model expressing the human Notch2 intracellular domain (hN2ICD) in cardiomyocytes (hN2ICD-Tg CM ) was generated and the mice spontaneously developed ventricular diastolic dysfunction with preserved ejection fraction and cardiac hypertrophy. Ectopic hN2ICD expression promoted cardiomyocyte hypertrophy by suppressing adenylosuccinate lyase (ADSL)-mediated adenosine 5′-monophosphate (AMP) generation, which further enhanced the activation of the mammalian target of rapamycin complex 1 pathway by reducing AMP-activated kinase activity. Hairy and enhancer of split 1 silencing abrogated hN2ICD-induced cardiomyocyte hypertrophy by increasing Adsl transcription. Importantly, pharmacological activation of AMP-activated kinase ameliorated cardiac hypertrophy and dysfunction in hN2ICD-Tg CM mice. The frameshift mutation in Notch2 exon 34 (c.6426dupT), which causes early-onset HCS, induces AC16 human cardiomyocyte hypertrophy through suppressing ADSL-mediated AMP generation. Thus, targeting Notch2-mediated purine nucleotide metabolism may be an attractive therapeutic approach to heart failure treatment. 
653 |a Enhancer-of-split protein 
653 |a Hypertrophy 
653 |a Adenylosuccinate lyase 
653 |a Nucleotides 
653 |a Kinases 
653 |a Cardiomyocytes 
653 |a TOR protein 
653 |a Transcription activation 
653 |a Heart diseases 
653 |a Frameshift mutation 
653 |a Congestive heart failure 
653 |a AMP 
653 |a Hereditary diseases 
653 |a Age 
653 |a Notch protein 
653 |a Metabolism 
653 |a Rapamycin 
653 |a Animal models 
653 |a Mutation 
653 |a Adenosine monophosphate 
700 1 |a Li, Yizhe 
700 1 |a Chen, Shihong 
700 1 |a Yu, Tingting 
700 1 |a Sun, Weiyan 
700 1 |a Liu, Jiao 
700 1 |a Ren, Huiwen 
700 1 |a Zhou, Yao 
700 1 |a Wang, Lu 
700 1 |a Tao, Xixi 
700 1 |a Du, Ronglu 
700 1 |a Shang, Wenlong 
700 1 |a Li, Yinxiu 
700 1 |a Tian, Danyang 
700 1 |a Wang, Bei 
700 1 |a Shen, Yujun 
700 1 |a Liu, Qian 
700 1 |a Yu, Ying 
773 0 |t Research  |g vol. 8 (2025) 
786 0 |d ProQuest  |t Science Database 
856 4 1 |3 Citation/Abstract  |u https://www.proquest.com/docview/3254941535/abstract/embedded/7BTGNMKEMPT1V9Z2?source=fedsrch 
856 4 0 |3 Full Text - PDF  |u https://www.proquest.com/docview/3254941535/fulltextPDF/embedded/7BTGNMKEMPT1V9Z2?source=fedsrch