Inheritance of certain alleles in the non-muscle heavy chain myosin gene MYH9 can result in a nephropathy. It is believed that MYH9 nephropathy may underly progressive nephrosclerosis in hypertensive Black Americans.


Gene affected: MYH9 (non-muscle heavy chain 9 myosin) on 22q11.2


MYH9 nephropathies include:

(1) sporadic, idiopathic focal segmental glomerulosclerosis (FSGS)

(2) focal global glomerulosclersosis (FGGS)

(3) collapsing glomerulopathy


Clinical features:

(1) hypertension

(2) some degree of proteinuria (may range from minimal to nephrotic syndrome levels)

(3) progression to renal failure (rapidly with collapsing glomerulopathy)


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