Evidence map›Paper›PMID 39809927›Full record

ArticleNature cardiovascular research2025

Correcting mitochondrial loss mitigates NOTCH1-related aortopathy in mice.

Yuyi Tang, Jingjing Zhang, Yixuan Fang, Kai Zhu, Jingqiao Zhu, Ce Huang, Zhuxin Xie, Shan Zhang, Wenrui Ma, Guoquan Yan and 16 more

Abstract read
PubMed Publisher
In one paragraph

Article in Nature cardiovascular research, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.

0numbers the graph read from it
0cells of the map it votes in
4citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

4 citing papers in PubMed.

  1. Review
  2. Review
  3. Review
  4. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

26 authors.

Yuyi Tang *Shanghai Fifth People's Hospital and Institutes of Biomedical Sciences Zhongshan Hospital, Shanghai Medical College, Fudan University, Shanghai, China.
Jingjing Zhang *Shanghai Fifth People's Hospital and Institutes of Biomedical Sciences Zhongshan Hospital, Shanghai Medical College, Fudan University, Shanghai, China.ORCID http://orcid.org/0009-0004-0099-1008
Yixuan Fang *Shanghai Fifth People's Hospital and Institutes of Biomedical Sciences Zhongshan Hospital, Shanghai Medical College, Fudan University, Shanghai, China.
Kai Zhu *Department of Cardiac Surgery, Zhongshan Hospital, Fudan University, Shanghai, China.ORCID http://orcid.org/0000-0002-2391-4193
Jingqiao ZhuShanghai Fifth People's Hospital and Institutes of Biomedical Sciences Zhongshan Hospital, Shanghai Medical College, Fudan University, Shanghai, China.
Ce HuangShanghai Institute of Infectious Disease and Biosecurity, Shanghai Medical College, Fudan University, Shanghai, China.
Zhuxin XieShanghai Fifth People's Hospital and Institutes of Biomedical Sciences Zhongshan Hospital, Shanghai Medical College, Fudan University, Shanghai, China.
Shan ZhangShanghai Fifth People's Hospital and Institutes of Biomedical Sciences Zhongshan Hospital, Shanghai Medical College, Fudan University, Shanghai, China.
Wenrui MaDepartment of Cardiac Surgery, Zhongshan Hospital, Fudan University, Shanghai, China.
Guoquan YanShanghai Fifth People's Hospital and Institutes of Biomedical Sciences Zhongshan Hospital, Shanghai Medical College, Fudan University, Shanghai, China.
Shaowen LiuShanghai Fifth People's Hospital and Institutes of Biomedical Sciences Zhongshan Hospital, Shanghai Medical College, Fudan University, Shanghai, China.
Xin LiuShanghai Fifth People's Hospital and Institutes of Biomedical Sciences Zhongshan Hospital, Shanghai Medical College, Fudan University, Shanghai, China.
Wenjing HanShanghai Fifth People's Hospital and Institutes of Biomedical Sciences Zhongshan Hospital, Shanghai Medical College, Fudan University, Shanghai, China.
Yue XinShanghai Fifth People's Hospital and Institutes of Biomedical Sciences Zhongshan Hospital, Shanghai Medical College, Fudan University, Shanghai, China.
Chenxi YangShanghai Fifth People's Hospital and Institutes of Biomedical Sciences Zhongshan Hospital, Shanghai Medical College, Fudan University, Shanghai, China.
Mieradilijiang AbudupataerDepartment of Cardiac Surgery, Zhongshan Hospital, Fudan University, Shanghai, China.
Peiyun ZhouShanghai Fifth People's Hospital and Institutes of Biomedical Sciences Zhongshan Hospital, Shanghai Medical College, Fudan University, Shanghai, China.
Chenxi HeShanghai Fifth People's Hospital and Institutes of Biomedical Sciences Zhongshan Hospital, Shanghai Medical College, Fudan University, Shanghai, China.
Hao LaiDepartment of Cardiac Surgery, Zhongshan Hospital, Fudan University, Shanghai, China.
Chunsheng WangDepartment of Cardiac Surgery, Zhongshan Hospital, Fudan University, Shanghai, China.
Yang LiuDepartment of Biochemistry and Molecular Biology, School of Basic Medical Sciences, Shanghai Medical College, Fudan University, Shanghai, China.
Fei LanShanghai Fifth People's Hospital and Institutes of Biomedical Sciences Zhongshan Hospital, Shanghai Medical College, Fudan University, Shanghai, China.
Dan YeShanghai Fifth People's Hospital and Institutes of Biomedical Sciences Zhongshan Hospital, Shanghai Medical College, Fudan University, Shanghai, China.
Fa-Xing YuShanghai Fifth People's Hospital and Institutes of Biomedical Sciences Zhongshan Hospital, Shanghai Medical College, Fudan University, Shanghai, China.ORCID http://orcid.org/0000-0003-4834-9335
Yanhui XuShanghai Fifth People's Hospital and Institutes of Biomedical Sciences Zhongshan Hospital, Shanghai Medical College, Fudan University, Shanghai, China.
Weijia ZhangShanghai Fifth People's Hospital and Institutes of Biomedical Sciences Zhongshan Hospital, Shanghai Medical College, Fudan University, Shanghai, China. weijiazhang@fudan.edu.cn.ORCID http://orcid.org/0000-0001-6928-0416

Funding

National Natural Science Foundation of China (National Science Foundation of China) 51927805National Natural Science Foundation of China (National Science Foundation of China) 82070482National Natural Science Foundation of China (National Science Foundation of China) 82200525National Natural Science Foundation of China (National Science Foundation of China) 82370472
6 · The paper itself

Abstract

Loss-of-function mutations in NOTCH1 were previously linked to thoracic aortopathy, a condition for which non-surgical treatment options are limited. Based on clinical proteome analysis, we hypothesized that mitochondrial fusion and biogenesis in aortic smooth muscle cells (SMCs) are crucial for regulating the progression of NOTCH1-related aortopathy. Here we demonstrate that SMC-specific Notch1 knockout mice develop aortic pathology, including stiffening, dilation and focal dissection. These changes are accompanied by decreased expression of MFN1/2 and TFAM, mirroring findings in human patients. SMC-specific deletion of Mfn1 and/or Mfn2 genes recapitulates the aortopathy seen in Notch1-deficient mice. Prophylactic or therapeutic approaches aimed at increasing mitochondrial DNA copy number, either through AAV-mediated overexpression of Mfn1/2 or oral treatment with mitofusion activators teriflunomide or leflunomide, help mitigate or slow the progression of aortopathy in SMC-Notch1

Indexed as

Aorta, ThoracicAortic Aneurysm, ThoracicAortic DiseasesAortic DissectionMitochondriaMitochondrial DynamicsMuscle, Smooth, VascularMyocytes, Smooth MuscleReceptor, Notch1AnimalsCells, CulturedDisease Models, AnimalDNA-Binding ProteinsDNA, MitochondrialGTP PhosphohydrolasesHigh Mobility Group ProteinsDNA-Binding ProteinsDNA, MitochondrialGTP PhosphohydrolasesHigh Mobility Group ProteinsLeflunomideMfn1 protein, mouseMfn2 protein, mouseNotch1 protein, mouseReceptor, Notch1Tfam protein, mouseTranscription Factors

Identifiers

PMID39809927

What Socratic holds

Textmetadata
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.