Evidence map›Paper›PMID 33230159›Full record

ArticleScientific reports2020

Quantitative proteomics reveal lineage-specific protein profiles in iPSC-derived Marfan syndrome smooth muscle cells.

Cristiana Iosef, Albert J Pedroza, Jason Z Cui, Alex R Dalal, Mamoru Arakawa, Yasushi Tashima, Tiffany K Koyano, Grayson Burdon, Samantha M P Churovich, Joshua O Orrick and 2 more

Open access · goldAbstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Scientific reports, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.

0numbers the graph read from it
0cells of the map it votes in
17citing papers in PubMed
1.4field-weighted citation impact, top 17% of its field
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

17 citing papers in PubMed, 20 citations in OpenAlex.

  1. Article
  2. Article
  3. Article
  4. Review
  5. Article
  6. Genetic analysis of a novelIntractable & rare diseases research · 2024
    Article
  7. Use of iPSC-Derived Smooth Muscle Cells to Model Physiology and Pathology.Arteriosclerosis, thrombosis, and vascular biology · 2024
    Review
  8. Human stem cell models for Marfan syndrome: aFrontiers in cell and developmental biology · 2024
    Review
  9. Review
  10. Review
  11. Review
  12. Article
  13. Article
  14. Article
  15. Review
  16. Review
  17. Transforming growth factor-beta (TGF-β) in the pathogenesis of hereditary thoracic aneurysm disorders.Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology
    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

12 authors at 1 institution in 1 country.

Cristiana IosefDepartment of Cardiothoracic Surgery, Stanford University, 300 Pasteur Dr, Falk CVRB, Stanford, CA, 94305, USA.
Albert J PedrozaDepartment of Cardiothoracic Surgery, Stanford University, 300 Pasteur Dr, Falk CVRB, Stanford, CA, 94305, USA.
Jason Z CuiDepartment of Cardiothoracic Surgery, Stanford University, 300 Pasteur Dr, Falk CVRB, Stanford, CA, 94305, USA.
Alex R DalalDepartment of Cardiothoracic Surgery, Stanford University, 300 Pasteur Dr, Falk CVRB, Stanford, CA, 94305, USA.
Mamoru ArakawaDepartment of Cardiothoracic Surgery, Stanford University, 300 Pasteur Dr, Falk CVRB, Stanford, CA, 94305, USA.
Yasushi TashimaDepartment of Cardiothoracic Surgery, Stanford University, 300 Pasteur Dr, Falk CVRB, Stanford, CA, 94305, USA.
Tiffany K KoyanoDepartment of Cardiothoracic Surgery, Stanford University, 300 Pasteur Dr, Falk CVRB, Stanford, CA, 94305, USA.
Grayson BurdonDepartment of Cardiothoracic Surgery, Stanford University, 300 Pasteur Dr, Falk CVRB, Stanford, CA, 94305, USA.
Samantha M P ChurovichDepartment of Cardiothoracic Surgery, Stanford University, 300 Pasteur Dr, Falk CVRB, Stanford, CA, 94305, USA.
Joshua O OrrickDepartment of Cardiothoracic Surgery, Stanford University, 300 Pasteur Dr, Falk CVRB, Stanford, CA, 94305, USA.
Mitchel ParianiDepartment of Pediatrics-Genetics, Stanford University, Stanford, CA, USA.
Michael P FischbeinDepartment of Cardiothoracic Surgery, Stanford University, 300 Pasteur Dr, Falk CVRB, Stanford, CA, 94305, USA. mfischbe@stanford.edu.
Stanford University · US

Funding

Endothelial-pericyte interactions in the pathogenesis of pulmonary arterial hypertensionR01HL139664 · NHLBI · STANFORD UNIVERSITY · PI DE JESUS PEREZ, VINICIO A · 2018 to 2025
$4.7M
Stanford Integrated Cardiovascular/Pulmonary Residency Research Training ProgramR38HL143615 · NHLBI · STANFORD UNIVERSITY · PI Michael Peter Fischbein, Marlene Rabinovitch · 2020 to 2026
$2.2M
Marfan Aortic Embryologic Origin Influences miR-29b Regulators and TargetsR01AR066629 · NIAMS · STANFORD UNIVERSITY · PI FISCHBEIN, MICHAEL PETER · 2016 to 2020
$1.5M
Stanford Undergraduate URM Summer Cardiovascular Research ProgramR25HL147666 · NHLBI · STANFORD UNIVERSITY · PI VINICIO A DE JESUS PEREZ, Mary Red-Horse · 2019 to 2026
$1.0M
NHLBI NIH HHS R01 HL139664NHLBI NIH HHS R25 HL147666NHLBI NIH HHS R38 HL143615NIAMS NIH HHS R01 AR066629
6 · The paper itself

Abstract

Marfan syndrome (MFS) is a connective tissue disorder caused by mutations in the FBN1 gene that produces wide disease phenotypic variability. The lack of ample genotype-phenotype correlation hinders translational study development aimed at improving disease prognosis. In response to this need, an induced pluripotent stem cell (iPSC) disease model has been used to test patient-specific cells by a proteomic approach. This model has the potential to risk stratify patients to make clinical decisions, including timing for surgical treatment. The regional propensity for aneurysm formation in MFS may be related to distinct smooth muscle cell (SMC) embryologic lineages. Thus, peripheral blood mononuclear cell (PBMC)-derived induced pluripotent stem cells (iPSC) were differentiated into lateral mesoderm (LM, aortic root) and neural crest (NC, ascending aorta/transverse arch) SMC lineages to model MFS aortic pathology. Isobaric Tags for Relative and Absolute Quantitation (iTRAQ) proteomic analysis by tandem mass spectrometry was applied to profile LM and NC iPSC SMCs from four MFS patients and two healthy controls. Analysis revealed 45 proteins with lineage-dependent expression in MFS patients, many of which were specific to diseased samples. Single protein-level data from both iPSC SMCs and primary MFS aortic root aneurysm tissue confirmed elevated integrin αV and reduced MRC2 in clinical disease specimens, validating the iPSC iTRAQ findings. Functionally, iPSC SMCs exhibited defective adhesion to a variety of extracellular matrix proteins, especially laminin-1 and fibronectin, suggesting altered cytoskeleton dynamics. This study defines the aortic embryologic origin-specific proteome in a validated iPSC SMC model to identify novel protein markers associated with MFS aneurysm phenotype. Translating iPSC findings into clinical aortic aneurysm tissue samples highlights the potential for iPSC-based methods to model MFS disease for mechanistic studies and therapeutic discovery in vitro.

Indexed as

AdipokinesAortaAortic Aneurysm, ThoracicCase-Control StudiesCell AdhesionCell DifferentiationCell LineageFemaleFibrillin-1FibronectinsGene Expression ProfilingGene Expression RegulationHumansInduced Pluripotent Stem CellsIntegrinsLamininAdipokinesFBN1 protein, humanFibrillin-1FibronectinsIntegrinsITGA5 protein, humanLamininlaminin 1Mannose-Binding LectinsMembrane GlycoproteinsMRC2 protein, humanReceptors, Cell Surface

Identifiers

PMID33230159
PMCPMC7683538
OpenAlexW3107506214

What OpenQuestion holds

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Registered trials

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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 OpenQuestion graph.