Fibulin-5/DANCE ELISA

Fibulin-5: Overview and Key Points

Introduction: Fibulin-5, also known as Developmental Arteries and Neural Crest EGF-like (DANCE), is a member of the fibulin protein family. This secreted glycoprotein is crucial for the formation and maintenance of elastic fibers in various tissues, playing a vital role in elastogenesis.

Molecular Characteristics:

• Structure: Fibulin-5 is a 66 kDa protein composed of 448 amino acids. It features an RGD (arginine-glycine-aspartic acid) motif in its N-terminal region, which is critical for its binding to integrins.
• Integrin Binding: The RGD motif allows Fibulin-5 to interact with integrins α5β1, αvβ3, and αvβ5, which are involved in cell adhesion and matrix interactions.

Biological Functions:

• Elastin Fiber Formation: Fibulin-5 is essential for the assembly of elastic fibers. It facilitates the formation of continuous elastin polymers and enhances the interaction between microfibrils and elastin, which is critical for the stability and organization of elastic fibers.
• Tissue Distribution: Fibulin-5 is prominently expressed in arteries during embryonic development and in various tissues including skin, lungs, and vasculature. Its expression increases in smooth muscle cells, fibroblasts, and endothelial cells in response to vascular injury.

Pathophysiological Relevance:

• Aging-Related Conditions: Mice deficient in Fibulin-5 exhibit conditions resembling human aging phenotypes, such as cutis laxa (loose skin), emphysema, and stiff arteries. These conditions result from the destruction and degradation of elastic fibers due to the absence of Fibulin-5, highlighting its critical role in maintaining elastic fiber integrity.
• Human Disease: The disruption of Fibulin-5 function in humans is associated with connective tissue disorders and may contribute to similar aging-related conditions observed in the mouse models.

Research and Clinical Implications:

• Elastogenesis Research: The role of Fibulin-5 in elastin fiber formation makes it a significant subject in studies focused on elastic fiber assembly and connective tissue disorders.
• Therapeutic Targets: Understanding the function and regulation of Fibulin-5 can provide insights into potential therapeutic approaches for treating diseases related to elastic fiber degradation and connective tissue abnormalities.

Summary: Fibulin-5 is a crucial protein for the formation and maintenance of elastic fibers, with a significant impact on the stability and organization of tissues such as skin, lungs, and blood vessels. Its role in elastogenesis and its association with aging-related conditions underline the importance of Fibulin-5 in both developmental biology and disease pathology.

This ELISA Kit from Tecan IBL International is for research use only.

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