Abstract: Type Ⅳ collagen is a vital component of basement membrane. Normal structure and function of type Ⅳ collagen is essential to maintain the integrity of glomerular filtration barrier. Mutations of type Ⅳ collagen genes cause a variety of kidney diseases, such as Alport Syndrome, thin basement membrane nephropathy, focal segmental glomerulosclerosis, and tubulointerstitial injury. Therefore, systematic analysis and clarification of the structure and function of type Ⅳ collagen, and the spectrum of kidney diseases caused by mutations in the type Ⅳ collagen genes play an important role in clinical diagnosis and treatment of these diseases.