True about fibronectin nephropathy are all except?
Ans. is 'a' i.e., Autosomal recessiveFibronectin nephropathy o Fibronectin nephropathy a disease of autosomal dominant mode of inheritance is characterized by massive glomerular accumulation of plasma fibronectin.o Fibronectin is a multifunctional, extracellular matrix glycoprotein that is active in cellular adhesion and migration. It is produced : -(i) Locally in the glomerulus by mesangial cells (cellular fibronectin).(ii) Liver synthesizes fibronectin that circulates (plasma fibronectin).o In fibronectin nephropathy, plasma fibronectin is accumulated.Pathological findingso The principal light microscopic change is glomerular enlargement and lobulation resulting from PAS and trichrome-positive mesangial deposits and mild mesangial proliferation. this process displaces the glomerular capillaries to the periphery of the lobule and decreases their luminal diameter. With Jones stain, the preserved GBM is usually at the periphery of the thickened capillary wall. There are no specific changes in renal tubules, interstitium or blood vessels.o By immunofluresence microscopy, the glomeruli do not consistently stain for immunoglobulin or complement components.o Immunohistological stain for fibronectin is strongly positive in the mesangial and subendothelial location.o The most consistent ultrastructural finding is large (giant), mesangial and subendothelial electron dense deposits that mirror the location of the PAS - positive fibronectin deposits.
Autosomal recessive inheritance
Gomerular enlargement and PAS+ trichrome mesangial deposit
Glomerulus do not consistently stain for Ig and complement
Ultrastructural feature is presence of large electron mesangial or subendothelial deposit