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| Status |
Public on May 16, 2012 |
| Title |
Epidermolysis bullosa associated cutaneous SCC fibroblast expression profiling |
| Organism |
Homo sapiens |
| Experiment type |
Expression profiling by array
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| Summary |
Patients with the genetic skin blistering disease recessive dystrophic epidermolysis bullosa (RDEB) develop aggressive cutaneous squamous cell carcinoma (cSCC). Metastasis leading to mortality is greater in RDEB than in other patient groups with cSCC. Here we investigate the dermal component in RDEB using mRNA expression profiling to compare cultured fibroblasts isolated from individuals without cSCC and directly from tumor matrix in RDEB and non-RDEB samples. While gene expression of RDEB normal skin fibroblasts resembled that of cancer-associated fibroblasts, RDEB cancer-associated fibroblasts exhibited a distinct and divergent gene expression profile, with a large proportion of the differentially expressed genes involved in matrix and cell adhesion. RDEB cancer-associated fibroblasts conferred increased adhesion and invasion to tumor and non-tumor keratinocytes. Reduction of COL7A1, the defective gene in RDEB, in normal dermal fibroblasts led to increased type XII collagen, thrombospondin-1 and Wnt-5A, while re-expression of wild type COL7A1 in RDEB fibroblasts decreased type XII collagen, thrombospondin- 1, and Wnt-5A expression, reduced tumor cell invasion in organotypic culture, and restricted tumor growth in vivo. Overall our findings demonstrate that matrix composition in patients with RDEB is a permissive environment for tumor development, and type VII collagen directly regulates the composition of matrix proteins secreted by dermal and cancer-associated fibroblasts.
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| Overall design |
16 samples
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| Contributor(s) |
South AP, Ng YZ |
| Citation(s) |
22564523 |
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| Submission date |
May 03, 2012 |
| Last update date |
Feb 22, 2018 |
| Contact name |
Andrew South |
| E-mail(s) |
[email protected]
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| Phone |
+44 1382 496432
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| Organization name |
University of Dundee
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| Department |
Division of Cancer Research
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| Street address |
Ninewells Hopsital and Medical School
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| City |
Dundee |
| ZIP/Postal code |
DD1 9SY |
| Country |
United Kingdom |
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| Platforms (1) |
| GPL4133 |
Agilent-014850 Whole Human Genome Microarray 4x44K G4112F (Feature Number version) |
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| Samples (16)
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| GSM926606 |
primary cultured normal human dermal fibroblasts NHF2 |
| GSM926607 |
primary cultured normal human dermal fibroblasts NHF66 |
| GSM926608 |
primary cultured normal human dermal fibroblasts NHF88 |
| GSM926609 |
primary cultured cSCC fibroblasts SCCT6F |
| GSM926610 |
primary cultured cSCC fibroblasts SCCIC15 |
| GSM926611 |
primary cultured cSCC fibroblasts SCCT7F |
| GSM926612 |
primary cultured cSCC fibroblasts SCCIC16 |
| GSM926613 |
primary cultured cSCC fibroblasts SCCIC17 |
| GSM926614 |
primary cultured RDEB dermal fibroblasts RDEB26F |
| GSM926615 |
primary cultured RDEB dermal fibroblasts RDEB27F |
| GSM926616 |
primary cultured RDEB dermal fibroblasts RDEB48F |
| GSM926617 |
primary cultured RDEB dermal fibroblasts RDEB23F |
| GSM926618 |
primary cultured RDEB cSCC fibroblasts RDEB2SCC1F |
| GSM926619 |
primary cultured RDEB cSCC fibroblasts RDEB3SCC1F |
| GSM926620 |
primary cultured RDEB cSCC fibroblasts RDEB43SCC1F |
| GSM926621 |
primary cultured RDEB cSCC fibroblasts RDEB7SCC2F |
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| Relations |
| BioProject |
PRJNA163255 |
| Supplementary file |
Size |
Download |
File type/resource |
| GSE37738_RAW.tar |
42.3 Mb |
(http)(custom) |
TAR (of TXT) |
| Processed data included within Sample table |
| Processed data provided as supplementary file |
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