In Polycystic Kidney Disease, which genes are typically associated with the autosomal dominant form?

In Polycystic Kidney Disease (PKD), the gene most commonly associated with the autosomal dominant form is PKD1. This gene encodes a protein called polycystin-1, which plays a crucial role in kidney function and the maintenance of normal tubular structure. Mutations in the PKD1 gene lead to the development of numerous cysts in the kidneys, causing progressive kidney enlargement and dysfunction over time.

The autosomal dominant form of PKD is typically characterized by a later onset of symptoms, often in adulthood, with varying degrees of renal impairment. While there are other genes involved in different forms of kidney disease, such as PKDH1 in a related but distinct condition and CFTR associated with cystic fibrosis, they do not pertain to the common autosomal dominant PKD. VWF (von Willebrand factor) is related to blood clotting disorders and is not associated with kidney cyst formation or PKD. Therefore, PKD1 is recognized as the most significant gene linked to the autosomal dominant type of Polycystic Kidney Disease.