Familial renal amyloidosis
Encyclopedia
Familial renal amyloidosis (or familial visceral amyloidosis, or hereditary amyloid nephropathy) is a form of amyloidosis
primarily presenting in the kidney
.
It is associated with fibrinogen alpha chain
, apolipoprotein A1
, and lysozyme
.
It is also known as "Ostertag" type, after B. Ostertag, who characterized it in 1932 and 1950.
Amyloidosis
In medicine, amyloidosis refers to a variety of conditions whereby the body produces "bad proteins", denoted as amyloid proteins, which are abnormally deposited in organs and/or tissues and cause harm. A protein is described as being amyloid if, due to an alteration in its secondary structure, it...
primarily presenting in the kidney
Kidney
The kidneys, organs with several functions, serve essential regulatory roles in most animals, including vertebrates and some invertebrates. They are essential in the urinary system and also serve homeostatic functions such as the regulation of electrolytes, maintenance of acid–base balance, and...
.
It is associated with fibrinogen alpha chain
Fibrinogen alpha chain
Fibrinogen alpha chain is a protein that in humans is encoded by the FGA gene.-Interactions:Fibrinogen alpha chain has been shown to interact with Tissue plasminogen activator.-Further reading:...
, apolipoprotein A1
Apolipoprotein A1
Apolipoprotein A-I is a protein that in humans is encoded by the APOA1 gene. It has a specific role in lipid metabolism.Apolipoprotein A-I is the major protein component of high density lipoprotein in plasma. Chylomicrons secreted from the intestinal enterocyte also contain ApoA1 but it is quickly...
, and lysozyme
Lysozyme
Lysozyme, also known as muramidase or N-acetylmuramide glycanhydrolase, are glycoside hydrolases, enzymes that damage bacterial cell walls by catalyzing hydrolysis of 1,4-beta-linkages between N-acetylmuramic acid and N-acetyl-D-glucosamine residues in a peptidoglycan and between...
.
It is also known as "Ostertag" type, after B. Ostertag, who characterized it in 1932 and 1950.