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Abnormal extracellular matrix deposition by fascial fibroblasts underlies the connective tissue pathology in the disease Radial Dysplasia

delete2026-02-27
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OA
AI
J
Jack D. Morgan
F
Ferheen Baig
J
Javier Barallobre‐Barreiro
F
Fursham Hamid
K
Karla Lozano Gonzalez
R
Rosamund Nuamah
S
Shichina Kannambath
P
Pawan Dhami
E
Edel DeBuitleir
G
G Murphy
B
Branavan Sivakumar
G
Gillian Smith
M
Manuel Mayr
M
Malcolm Logan
DOI:10.1016/j.matbio.2026.02.004delete
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Abstract

Abstract

En 中文
• Fascial fibroblast matrisome changes are present in a human limb birth defect. • Matrix composition changes cause disruption of material properties of fascia in human disease. • Fascial fibroblasts are a heterogeneous cell population, altered in human disease. • IL6 restores normal matrix composition and cell behaviour of disease fascial fibroblasts
Keywords:
fascial fibroblasts
extracellular matrix
radial dysplasia
matrix composition
IL6
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Journal

Matrix Biology cover
Matrix Biology
IF:
4.8
Papers:
109
Citations:
8.1K

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K
King's College London
Scholars:
1.2K
Papers: 564
Citations: 6.7W
G
Guy
Scholars:
17
Papers: 4
Citations: 0
G
Great Ormond Street Hospital for Children
Scholars:
135
Papers: 74
Citations: 1
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