Key Product Details

Species Reactivity

Human

Applications

ELISA

Label

Janelia Fluor 549

Antibody Source

Polyclonal Goat IgG

Concentration

Please see the vial label for concentration. If unlisted please contact technical services.

View all Collagen III alpha 1/COL3A1 Antibodies »

Product Specifications

Immunogen

This Collagen III alpha 1/COL3A1 Antibody was developed by hyperimmunizing goats with human type III collagen.

Specificity

Reacts with conformational determinants on human type III collagen as demonstrated by ELISA. May react with type III collagen from other species. Exhibits

Clonality

Polyclonal

Host

Goat

Isotype

IgG

Theoretical MW

139 kDa.
Disclaimer note: The observed molecular weight of the protein may vary from the listed predicted molecular weight due to post translational modifications, post translation cleavages, relative charges, and other experimental factors.

Applications for Collagen III alpha 1/COL3A1 Antibody [Janelia Fluor® 549]

Application
Recommended Usage

ELISA

Optimal dilutions of this antibody should be experimentally determined.
Application Notes
Optimal dilution of this antibody should be experimentally determined.

Formulation, Preparation, and Storage

Purification

Immunogen affinity purified

Formulation

50mM Sodium Borate

Preservative

0.05% Sodium Azide

Concentration

Please see the vial label for concentration. If unlisted please contact technical services.

Shipping

The product is shipped with polar packs. Upon receipt, store it immediately at the temperature recommended below.

Stability & Storage

Store at 4C in the dark.

Background: Collagen III alpha 1/COL3A1

Collagen III alpha 1, also referred to as collagen type III alpha 1 or COL3A1 for short, was first described in 1971 and is a member of the collagen superfamily and encoded COL3A1 gene (1, 2). In general, collagen III is an extracellular matrix protein that is synthesized as a preprocollagen followed by cleaving of the signal peptide to form the procollagen (1). The human COL3A1 gene is located on chromosome 2q32.2 and collagen III is synthesized as a homotrimer consisting of three identical alpha procollagen chains which are stabilized by disulfide bonds (1,2,3). Each alpha chain is 1466 amino acids (aa) in length with a theoretical molecular weight of 139 kDa for a single alpha chain (1). Structurally, each alpha chain is a left-handed helix which then join together to form a right-handed triple helix (1,2). C-terminal and N-terminal proteinases remove the globular ends of the procollagen to form the type III collagen (1).

Collagen III is a fibrillar collagen that constitutes 5-20% of all collagen in the body (1). It provides structural integrity and is found in many hallow organs and soft connective tissue including the vascular system, skin, lung, uterus, and intestine (1,2). Additionally, collagen III has be found to be associated with type I collagen in the same fibrils (1). Collagen III interacts with signaling integrins to carry out other key functions including cell adhesion, proliferation, and differentiation (1).

Mutations in the COL3A1 gene has been associated with a variety of human diseases, the most well-known being a group of connective tissue disorders termed Ehlers-Danlos Syndromes (1,2,4). Vascular Ehlers-Danlos Syndrome is a specific subtype that is considered the most severe and although the clinical manifestations vary, symptoms include thin skin and fragile blood vessels and can often result in both lung and heart complications (1,4). COL3A1 is also associated with glomerulopathies, or diseases of the glomeruli, which are characterized by an abundance of extracellular matrix (3). Collagenofibrotic glomerulopathy is one specific rare renal disease that is characterized by excessive levels of collagen III (3).

References

1. Kuivaniemi, H., & Tromp, G. (2019). Type III collagen (COL3A1): Gene and protein structure, tissue distribution, and associated diseases. Gene. https://doi.org/10.1016/j.gene.2019.05.003

2. Ricard-Blum S. (2011). The collagen family. Cold Spring Harbor perspectives in biology. https://doi.org/10.1101/cshperspect.a004978

3. Cohen A. H. (2012). Collagen Type III Glomerulopathies. Advances in chronic kidney disease. https://doi.org/10.1053/j.ackd.2012.02.017

4. Olson, S. L., Murray, M. L., & Skeik, N. (2019). A Novel Frameshift COL3A1 Variant in Vascular Ehlers-Danlos Syndrome. Annals of vascular surgery. https://doi.org/10.1016/j.avsg.2019.05.057

Alternate Names

COL3A1, EDS4A

Gene Symbol

COL3A1

Additional Collagen III alpha 1/COL3A1 Products

Product Documents for Collagen III alpha 1/COL3A1 Antibody [Janelia Fluor® 549]

Product Datasheets

Certificate of Analysis

To download a Certificate of Analysis, please enter a lot number in the search box below.

Download SDS

Product Specific Notices for Collagen III alpha 1/COL3A1 Antibody [Janelia Fluor® 549]



Sold under license from the Howard Hughes Medical Institute, Janelia Research Campus.

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

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