COL3A1 (E8D7R) XP® Rabbit mAb (Alexa Fluor® 555 Conjugate) #76735
- IHC
Supporting Data
| REACTIVITY | H |
| SENSITIVITY | Endogenous |
| MW (kDa) | |
| Source/Isotype | Rabbit IgG |
Application Key:
- IHC-Immunohistochemistry
Species Cross-Reactivity Key:
- H-Human
Product Information
Product Description
Product Usage Information
| Application | Dilution |
|---|---|
| Immunohistochemistry (Paraffin) | 1:50 - 1:200 |
Storage
Protocol
Specificity / Sensitivity
Species Reactivity:
Source / Purification
Background
Collagen 3 alpha 1 (COL3A1) is a major fibrillar collagen composed of three identical alpha-1 chains. It is present in most soft tissues, along with COL1A1, and is particularly high in tissues exhibiting elastic properties, such as the cardiac arterial wall and skin (4). Heterozygous mutations in the COL3A1 gene that cause missense mutation of a critical glycine residue in the triple helical domain of the alpha-1 chain result in vascular Ehlers-Danlos syndrome (vEDS). This mutation interferes with the ability of the alpha-1 chain to form collagen fibrils and thus disrupts macromolecular assembly of collagen fibers. vEDS is a severe and life-threatening disease as patients have a propensity for rupture of large arteries (4).
Increased amounts of type III COL3A1 are found in many fibrotic conditions, such as lung, liver, kidney fibrosis, and systemic sclerosis (5,6).
- Barkan, D. et al. (2010) Eur J Cancer 46, 1181-8.
- Hynes, R.O. (2009) Science 326, 1216-9.
- Ricard-Blum, S. (2011) Cold Spring Harb Perspect Biol 3, a004978.
- Kuivaniemi, H. and Tromp, G. (2019) Gene 707, 151-71.
- Karsdal, M.A. et al. (2017) Adv Drug Deliv Rev 121, 43-56.
- Ricard-Blum, S. et al. (2018) Matrix Biol 68-9, 122-49.
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