COL4A1 is a component of type IV collagen, primarily found in basement membranes of various tissues, including kidneys, lungs, and blood vessels. COL4A1 play a significant role in tissue homeostasis and organ function. Mutations in COL4A1 can lead to various disorders, including vascular and kidney diseases, highlighting COL4A1′s importance in health and disease. The extensive family of collagen proteins, including COL4A1, features a unique triple helix structure providing tensile strength and stability to the extracellular matrix. This structural feature enables complex connective tissues to withstand mechanical stress while facilitating cell adhesion and communication.