Evidence map›Paper›PMID 26711913›Full record

ArticleCell and tissue research2016

Forelimb contractures and abnormal tendon collagen fibrillogenesis in fibulin-4 null mice.

Dessislava Z Markova, Te-Cheng Pan, Rui-Zhu Zhang, Guiyun Zhang, Takako Sasaki, Machiko Arita, David E Birk, Mon-Li Chu

Abstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Cell and tissue research, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.

0numbers the graph read from it
0cells of the map it votes in
5citing 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

5 citing papers in PubMed.

  1. Article
  2. Review
  3. Article
  4. Review
  5. The "other" 15-40%: The Role of Non-Collagenous Extracellular Matrix Proteins and Minor Collagens in Tendon.Journal of orthopaedic research : official publication of the Orthopaedic Research Society · 2020
    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

8 authors.

Dessislava Z MarkovaDepartment of Dermatology and Cutaneous Biology, Sidney Kimmel Medical College, Thomas Jefferson University, 233 South 10th Street, Philadelphia, PA 19107, USA.
Te-Cheng PanDepartment of Dermatology and Cutaneous Biology, Sidney Kimmel Medical College, Thomas Jefferson University, 233 South 10th Street, Philadelphia, PA 19107, USA.
Rui-Zhu ZhangDepartment of Dermatology and Cutaneous Biology, Sidney Kimmel Medical College, Thomas Jefferson University, 233 South 10th Street, Philadelphia, PA 19107, USA.
Guiyun ZhangDepartment of Pathology, Anatomy and Cell Biology, Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA 19107, USA.
Takako SasakiDepartment of Biochemistry, Faculty of Medicine, Oita University, Oita, Japan.
Machiko AritaDepartment of Dermatology and Cutaneous Biology, Sidney Kimmel Medical College, Thomas Jefferson University, 233 South 10th Street, Philadelphia, PA 19107, USA.
David E BirkDepartment of Molecular Pharmacology & Physiology, Morsani College of Medicine, University of South Florida, Tampa, FL 33612, USA.
Mon-Li ChuDepartment of Dermatology and Cutaneous Biology, Sidney Kimmel Medical College, Thomas Jefferson University, 233 South 10th Street, Philadelphia, PA 19107, USA. mon-li.chu@jefferson.edu.

Funding

X-Ray Crystallography and Macromolecular CharacterizationP30CA056036 · NCI · THOMAS JEFFERSON UNIVERSITY · PI DANIEL P SILVER · 1995 to 2026
$94.8M
Regulated Assembly of the Tendon Extracellular MatrixR01AR044745 · NIAMS · UNIVERSITY OF SOUTH FLORIDA · PI BIRK, DAVID E · 1997 to 2016
$5.0M
Function of FibulinsR01GM055625 · NIGMS · THOMAS JEFFERSON UNIVERSITY · PI CHU, MON-LI H. · 1997 to 2004
$1.4M
NCI NIH HHS P30 CA056036NIAMS NIH HHS R01 AR044745NIGMS NIH HHS R01 GM055625
6 · The paper itself

Abstract

Fibulin-4 is an extracellular matrix glycoprotein essential for elastic fiber formation. Mice deficient in fibulin-4 die perinatally because of severe pulmonary and vascular defects associated with the lack of intact elastic fibers. Patients with fibulin-4 mutations demonstrate similar defects, and a significant number die shortly after birth or in early childhood from cardiopulmonary failure. The patients also demonstrate skeletal and other systemic connective tissue abnormalities, including joint laxity and flexion contractures of the wrist. A fibulin-4 null mouse strain was generated and used to analyze the roles of fibulin-4 in tendon fibrillogenesis. This mouse model displayed bilateral forelimb contractures, in addition to pulmonary and cardiovascular defects. The forelimb and hindlimb tendons exhibited disruption in collagen fibrillogenesis in the absence of fibulin-4 as analyzed by transmission electron microscopy. Fewer fibrils were assembled, and fibrils were disorganized compared with wild-type controls. The organization of developing tenocytes and compartmentalization of the extracellular space was also disrupted. Fibulin-4 was co-localized with fibrillin-1 and fibrillin-2 in limb tendons by using immunofluorescence microscopy. Thus, fibulin-4 seems to play a role in regulating tendon collagen fibrillogenesis, in addition to its essential function in elastogenesis.

Indexed as

AnimalsCollagenContractureExtracellular Matrix ProteinsFibrillinsForelimbHerniaPhenotypeProtein TransportTendonsCollagenEFEMP2 protein, humanExtracellular Matrix ProteinsFibrillinsCollagen fibrillogenesisCutis laxaElastic fiberFibrillinTendon

Identifiers

PMID26711913
PMCPMC4876057

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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.