Dataset Information
Secretome analysis of mouse fibroblast
Ontology highlight
ABSTRACT:
ORGANISM(S): Mus Musculus (mouse)
SUBMITTER: Sumio Ohtsuki
PROVIDER: PXD052580 | JPOST Repository | Fri Apr 11 00:00:00 BST 2025
REPOSITORIES: jPOST
Dataset's files
| Action | DRS | |||
|---|---|---|---|---|
| 10_H6989homo.wiff | Wiff | |||
| 10_H6989homo.wiff.scan | Wiff | |||
| 11_H6991homo.wiff | Wiff | |||
| 11_H6991homo.wiff.scan | Wiff | |||
| 12_H7112homo.wiff | Wiff |
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Publications
Fibrillin-1 G234D mutation in the hybrid1 domain causes tight skin associated with dysregulated elastogenesis and increased collagen cross-linking in mice.
Matrix biology : journal of the International Society for Matrix Biology 20241128
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<sup>G234D/G234D</sup> mice carrying a corresponding variant of FBN1 in the hybrid1 domain that was ide ...[more]
