Evidence map›Paper›PMID 38542525›Full record

ArticleInternational journal of molecular sciences2024

Modeling Lysosomal Storage Disorders in an Innovative Way: Establishment and Characterization of Stem Cell Lines from Human Exfoliated Deciduous Teeth of Mucopolysaccharidosis Type II Patients.

Sofia Carvalho, Juliana Inês Santos, Luciana Moreira, Ana Joana Duarte, Paulo Gaspar, Hugo Rocha, Marisa Encarnação, Diogo Ribeiro, Matilde Barbosa Almeida, Mariana Gonçalves and 11 more

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

Article in International journal of molecular sciences, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed
0.7field-weighted citation impact, top 32% 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

2 citing papers in PubMed, 2 citations in OpenAlex.

  1. Article
  2. Article
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

21 authors at 6 institutions in 1 country.

Sofia CarvalhoResearch and Development Unit, Department of Human Genetics, National Institute of Health Doutor Ricardo Jorge, INSA I.P., Rua Alexandre Herculano, 321, 4000-055 Porto, Portugal.
Juliana Inês SantosResearch and Development Unit, Department of Human Genetics, National Institute of Health Doutor Ricardo Jorge, INSA I.P., Rua Alexandre Herculano, 321, 4000-055 Porto, Portugal.
Luciana MoreiraResearch and Development Unit, Department of Human Genetics, National Institute of Health Doutor Ricardo Jorge, INSA I.P., Rua Alexandre Herculano, 321, 4000-055 Porto, Portugal.
Ana Joana DuarteResearch and Development Unit, Department of Human Genetics, National Institute of Health Doutor Ricardo Jorge, INSA I.P., Rua Alexandre Herculano, 321, 4000-055 Porto, Portugal.ORCID 0000-0001-6005-7322
Paulo GasparNewborn Screening, Metabolism and Genetics Unit, Department of Human Genetics, National Institute of Health Doutor Ricardo Jorge, INSA I.P., Rua Alexandre Herculano, 321, 4000-055 Porto, Portugal.ORCID 0000-0002-4255-0946
Hugo RochaNewborn Screening, Metabolism and Genetics Unit, Department of Human Genetics, National Institute of Health Doutor Ricardo Jorge, INSA I.P., Rua Alexandre Herculano, 321, 4000-055 Porto, Portugal.ORCID 0000-0001-5447-615X
Marisa EncarnaçãoResearch and Development Unit, Department of Human Genetics, National Institute of Health Doutor Ricardo Jorge, INSA I.P., Rua Alexandre Herculano, 321, 4000-055 Porto, Portugal.ORCID 0000-0002-3726-2851
Diogo RibeiroResearch and Development Unit, Department of Human Genetics, National Institute of Health Doutor Ricardo Jorge, INSA I.P., Rua Alexandre Herculano, 321, 4000-055 Porto, Portugal.ORCID 0000-0002-4681-0354
Matilde Barbosa AlmeidaResearch and Development Unit, Department of Human Genetics, National Institute of Health Doutor Ricardo Jorge, INSA I.P., Rua Alexandre Herculano, 321, 4000-055 Porto, Portugal.ORCID 0009-0006-6079-4780
Mariana GonçalvesResearch and Development Unit, Department of Human Genetics, National Institute of Health Doutor Ricardo Jorge, INSA I.P., Rua Alexandre Herculano, 321, 4000-055 Porto, Portugal.ORCID 0000-0002-3111-4612
Hugo DavidResearch and Development Unit, Department of Human Genetics, National Institute of Health Doutor Ricardo Jorge, INSA I.P., Rua Alexandre Herculano, 321, 4000-055 Porto, Portugal.ORCID 0000-0002-9623-4017
Liliana MatosResearch and Development Unit, Department of Human Genetics, National Institute of Health Doutor Ricardo Jorge, INSA I.P., Rua Alexandre Herculano, 321, 4000-055 Porto, Portugal.
Olga AmaralResearch and Development Unit, Department of Human Genetics, National Institute of Health Doutor Ricardo Jorge, INSA I.P., Rua Alexandre Herculano, 321, 4000-055 Porto, Portugal.
Luísa DiogoCentro de Referência de Doenças Hereditárias do Metabolismo do Centro Hospitalar Universitário de Coimbra, CR-DHM (CHUC), Praceta Professor Mota Pinto, 3004-561 Coimbra, Portugal.ORCID 0000-0003-3821-6643
Sara FerreiraCentro de Referência de Doenças Hereditárias do Metabolismo do Centro Hospitalar Universitário de Coimbra, CR-DHM (CHUC), Praceta Professor Mota Pinto, 3004-561 Coimbra, Portugal.ORCID 0000-0002-5462-5757
Constança SantosCentro de Referência de Doenças Hereditárias do Metabolismo do Centro Hospitalar Universitário de Coimbra, CR-DHM (CHUC), Praceta Professor Mota Pinto, 3004-561 Coimbra, Portugal.
Esmeralda MartinsCentro Hospitalar Universitário do Porto, Hospital de Santo António, CHPorto, Largo do Prof. Abel Salazar, 4099-001 Porto, Portugal.
Maria João PrataBiology Department, Faculty of Sciences, University of Porto, Rua do Campo Alegre, 4169-007 Porto, Portugal.ORCID 0000-0002-0583-1028
Luís Pereira de AlmeidaFaculty of Pharmacy, University of Coimbra, Polo das Ciências da Saúde, Azinhaga de Santa Comba, 3000-548 Coimbra, Portugal.
Sandra AlvesResearch and Development Unit, Department of Human Genetics, National Institute of Health Doutor Ricardo Jorge, INSA I.P., Rua Alexandre Herculano, 321, 4000-055 Porto, Portugal.ORCID 0000-0002-8881-9197
Maria Francisca CoutinhoResearch and Development Unit, Department of Human Genetics, National Institute of Health Doutor Ricardo Jorge, INSA I.P., Rua Alexandre Herculano, 321, 4000-055 Porto, Portugal.ORCID 0000-0002-2222-3622
Universidade do Porto · PTHospitais da Universidade de Coimbra · PTNational Institute of Health Dr. Ricardo Jorge · PTHospital de Santo António · PTUniversity of Coimbra · PTUniversity of Trás-os-Montes and Alto Douro · PT

Funding

Fundação para a Ciência e Tecnologia http://doi.org/10.54499/EXPL/BTM-SAL/0659/2021Portuguese Society for Metabolic Disorder, SPDM Bolsa SPDM de apoio à investigação Dr. Aguinaldo Cabral 2018 (2019DGH1629/ SPDM2018I&DSanfilippo Children's Foundation 2019DGH1656/ SCF2019I&D
6 · The paper itself

Abstract

Among the many lysosomal storage disorders (LSDs) that would benefit from the establishment of novel cell models, either patient-derived or genetically engineered, is mucopolysaccharidosis type II (MPS II). Here, we present our results on the establishment and characterization of two MPS II patient-derived stem cell line(s) from deciduous baby teeth. To the best of our knowledge, this is the first time a stem cell population has been isolated from LSD patient samples obtained from the dental pulp. Taking into account our results on the molecular and biochemical characterization of those cells and the fact that they exhibit visible and measurable disease phenotypes, we consider these cells may qualify as a valuable disease model, which may be useful for both pathophysiological assessments and in vitro screenings. Ultimately, we believe that patient-derived dental pulp stem cells (DPSCs), particularly those isolated from human exfoliated deciduous teeth (SHEDs), may represent a feasible alternative to induced pluripotent stem cells (iPSCs) in many labs with standard cell culture conditions and limited (human and economic) resources.

Indexed as

Lysosomal Storage DiseasesMucopolysaccharidosis IICell DifferentiationCell LineCell ProliferationDental PulpHumansLysosomesStem CellsTooth, Deciduousdental pulp stem cells (DPSCs)disease modelinginduced pluripotent stem cells (iPSCs)in vitro modelsmucopolysaccharidosis type IIstem cells from human exfoliated deciduous teeth (SHEDs)

Identifiers

PMID38542525
PMCPMC10970756
OpenAlexW4393039647

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.