type v collagen
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2021 ◽  
Author(s):  
Keren Machol ◽  
Urszula Polak ◽  
Monika Weisz-Hubshman ◽  
I-Wen Song ◽  
Shan Chen ◽  
...  

Abstract Type V collagen is a regulatory fibrillar collagen essential for type I collagen fibril nucleation and organization and its deficiency leads to structurally abnormal extracellular matrix. Haploinsufficiency of the Col5a1 gene encoding α(1) chain of type V collagen is the primary cause of classic Ehlers Danlos Syndrome (EDS). The mechanisms by which this initial insult leads to the spectrum of clinical presentation is not fully understood. Using transcriptome analysis of skin and Achilles tendons from Col5a1 haploinsufficient (Col5a1+/−) mice, we recognized molecular alterations associated with the tissue phenotypes. We identified dysregulation of extracellular matrix components including thrombospondin-1, lysyl oxidase, and lumican in the skin of Col5a1+/− mice when compared to control. We also identified upregulation of Tgf-β in serum and increased expression of pSmad2 in skin from Col5a1+/− mice suggesting Tgf-β dysregulation as a contributor for abnormal wound healing and atrophic scaring seen in classic EDS. Together, these findings support altered matrix to cell signaling as a component of the pathogenesis of the tissue phenotype in classic EDS and point out potential downstream signaling pathways that may be targeted for treatment of the disease.


2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Hsing-Fang Tsai ◽  
Yu-Chan Chang ◽  
Chien-Hsiu Li ◽  
Ming-Hsien Chan ◽  
Chi-Long Chen ◽  
...  

AbstractGlioblastoma (GBM) is a fatal cancer. Existing therapies do not have significant efficacy for GBM patients. Previous studies have shown that the collagen family is involved in the regulation of the extracellular environment of cancer cells, and these conditions could become an important factor for effective treatment. Therefore, we screened various collagen types and observed that the type V collagen α1 chain (COL5A1) gene plays a pivotal role in GBM. We further examined whether the overexpression of COL5A1 is common in mesenchymal subtypes and is related to the survival rate of GBM patients through several in silico cohorts. In addition, our cohort also showed a consistent trend in COL5A1 protein levels. Most importantly, we validated the cell mobility, metastatic ability and actin polymerization status caused by COL5A1 with two-way models. Based on these results, we established a transcriptomics dataset based on COL5A1. Moreover, PPRC1, GK and ESM1 were predicted by ingenuity pathway analysis (IPA) to be transcription factors or to participate downstream. We investigated the involvement of COL5A1 in extracellular remodeling and the regulation of actin filaments in the metastasis of GBM. Our results indicate that the COL5A1−PPRC1−ESM1 axis may represent a novel therapeutic target in GBM.


2021 ◽  
Author(s):  
Prashant Chandrasekaran ◽  
Bryan Kwok ◽  
Biao Han ◽  
Sheila M. Adams ◽  
Chao Wang ◽  
...  

2021 ◽  
Vol 58 (2) ◽  
pp. 53-64
Author(s):  
Ikuyo NAKAJIMA ◽  
Fumio NAKAMURA ◽  
Koichi CHIKUNI

2021 ◽  
Vol 220 ◽  
pp. 153382
Author(s):  
Roberta Goncalves Marangoni ◽  
Benjamin D. Korman ◽  
Edwin R. Parra ◽  
Ana Paula P. Velosa ◽  
Hermes V. Barbeiro ◽  
...  

2020 ◽  
Vol 216 (12) ◽  
pp. 153277
Author(s):  
Marcelo Luiz Balancin ◽  
Walcy Rosolia Teodoro ◽  
Camila Machado Baldavira ◽  
Tabatha Gutierrez Prieto ◽  
Cecilia Farhat ◽  
...  

Cell ◽  
2020 ◽  
Vol 182 (3) ◽  
pp. 545-562.e23 ◽  
Author(s):  
Tomohiro Yokota ◽  
Jackie McCourt ◽  
Feiyang Ma ◽  
Shuxun Ren ◽  
Shen Li ◽  
...  

2020 ◽  
Vol 17 (9) ◽  
pp. 539-539
Author(s):  
Gregory B. Lim
Keyword(s):  

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