X-Nico

4 unusual facts about Alport syndrome


Alport syndrome

Gradual progression to ESRD in the index case of at least two family members.

COL4A4

Mutations in this gene are associated with type II autosomal recessive Alport syndrome (hereditary glomerulonephropathy) and with familial benign hematuria (thin basement membrane disease).

COL4A5

Mutations in this gene are associated with X-linked Alport syndrome, also known as hereditary nephritis.

COL4A6

Deletions in the alpha 5 gene that extend into the alpha 6 gene result in diffuse leiomyomatosis accompanying the X-linked Alport syndrome caused by the deletion in the alpha 5 gene.



see also