Evidence map›Paper›PMID 39615636›Full record

ArticleMatrix biology : journal of the International Society for Matrix Biology2025

Fibrillin-1 G234D mutation in the hybrid1 domain causes tight skin associated with dysregulated elastogenesis and increased collagen cross-linking in mice.

Asm Sakhawat Hossain, Maria Thea Rane Dela Cruz Clarin, Kenichi Kimura, George Biggin, Yuki Taga, Koichiro Uto, Ayana Yamagishi, Eri Motoyama, Narenmandula, Kazunori Mizuno and 8 more

Abstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Matrix biology : journal of the International Society for Matrix Biology, 2025. 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
–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

2 citing papers in PubMed.

  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

18 authors.

Asm Sakhawat HossainGraduate School of Comprehensive Human Sciences, University of Tsukuba, Japan; Tsukuba Advanced Research Alliance (TARA), Life Science Center for Survival Dynamics, University of Tsukuba, Japan; Department of Pharmacy, Varendra University, Bangladesh.
Maria Thea Rane Dela Cruz ClarinSchool of Integrative and Global Major, University of Tsukuba, Japan; Tsukuba Advanced Research Alliance (TARA), Life Science Center for Survival Dynamics, University of Tsukuba, Japan; National Institute for Material Science, Japan.
Kenichi KimuraTsukuba Advanced Research Alliance (TARA), Life Science Center for Survival Dynamics, University of Tsukuba, Japan.
George BigginDivision of Cell Matrix Biology and Regenerative Medicine, Faculty of Biology, Medicine and Health, Wellcome Trust Centre for Cell-Matrix Research, University of Manchester, UK.
Yuki TagaNippi Research Institute of Biomatrix, Japan.
Koichiro UtoNational Institute for Material Science, Japan.
Ayana YamagishiNational Institute of Advanced Industrial Science and Technology, Japan.
Eri MotoyamaTsukuba Advanced Research Alliance (TARA), Life Science Center for Survival Dynamics, University of Tsukuba, Japan.
NarenmandulaGraduate School of Comprehensive Human Sciences, University of Tsukuba, Japan; Tsukuba Advanced Research Alliance (TARA), Life Science Center for Survival Dynamics, University of Tsukuba, Japan.
Kazunori MizunoNippi Research Institute of Biomatrix, Japan.
Chikashi NakamuraNational Institute of Advanced Industrial Science and Technology, Japan.
Keiichi AsanoTsukuba Advanced Research Alliance (TARA), Life Science Center for Survival Dynamics, University of Tsukuba, Japan.
Sumio OhtsukiDepartment of Pharmaceutical Microbiology, Faculty of Life Sciences, Kumamoto University, Japan.
Tomoyuki NakamuraDepartment of Pharmacology, Kansai Medical University, Japan.
Sachiko KankiDepartment of Surgery, Osaka Medical and Pharmaceutical University, Japan.
Clair BaldockDivision of Cell Matrix Biology and Regenerative Medicine, Faculty of Biology, Medicine and Health, Wellcome Trust Centre for Cell-Matrix Research, University of Manchester, UK.
Erna RajaTsukuba Advanced Research Alliance (TARA), Life Science Center for Survival Dynamics, University of Tsukuba, Japan. Electronic address: rajaerna@tara.tsukuba.ac.jp.
Hiromi YanagisawaTsukuba Advanced Research Alliance (TARA), Life Science Center for Survival Dynamics, University of Tsukuba, Japan. Electronic address: hkyanagisawa@tara.tsukuba.ac.jp.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Fibrillin-1, an extracellular matrix (ECM) protein encoded by the FBN1 gene, serves as a microfibril scaffold crucial for elastic fiber formation and homeostasis in pliable tissue such as the skin. Aside from causing Marfan syndrome, some mutations in FBN1 result in scleroderma, marked by hardened and thicker skin which limits joint mobility. Here, we describe a tight skin phenotype in the Fbn1

Indexed as

CollagenElastic TissueFibrillin-1SkinAdipokinesAnimalsExtracellular MatrixFibroblastsHumansMaleMarfan SyndromeMiceMicrofibrilsMutationPhenotypeAdipokinesCollagenFBN1 protein, humanFbn1 protein, mouseFibrillin-1Collagen cross-linkingElastic fibersExtracellular matrixFasciaFibrillin-1FibroblastsTight skin

Identifiers

PMID39615636
PMCPMC11747857

What OpenQuestion holds

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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 OpenQuestion graph.