Inflammatory Amyloidosis
Amyloidosis is a group of familial and/or inherited, degenerative, and infectious disease processes, characterized by the abnormal protein folding and deposition of a complex substance composed of amyloid protein and fibrils.
Amyloid A amyloidosis (AA) is the most common form of systemic amyloidosis worldwide. It is characterized by extracellular tissue deposition of fibrils that are composed of fragments of serum amyloid A (SAA) protein, a major acute-phase reactant protein, produced mainly by liver cells (hepatocytes). It occurs in the course of a chronic inflammatory disease of either infectious or noninfectious origin, hereditary periodic fevers, and with certain neoplasms such as Hodgkin disease and renal cell carcinoma.1
Glomerular amyloidosis usually causes protein in the urine. Amyloidosis is sometimes inherited in the Chinese Shar-Pei.2
Different Types of Amyloid Protein Are Involved in Amyloidosis
There are several types of amyloid, and the classification of amyloidosis is based on which amyloid protein is involved. Amyloid is now classified chemically. The amyloidoses (collective name for diseases of amyloidosis type) are referred to with a capital A (for amyloid) followed by an abbreviation for the fibril protein. Twenty-three different fibril proteins are described in human amyloidosis with variable clinical features. In all forms of amyloidosis, the cell secretes the precursor protein in a soluble form that becomes insoluble at some tissue site, compromising organ function.
Conditions Associated with Amyloidosis
References:
1. Mohammed Mubashir Ahmed, MD. Amyloidosis, AA (Inflammatory)
2. Cynthia M. Khan, BA, MA and Scott Line, DVM, PdD, Dipl ACVB. The Merck/Merial Manual for Pet Health Home Edition. Whitehouse Station, NJ: Merck & Co., Inc., 2007: 294, 307
3. Bruce A Baethge, MD. "Amyloidosis, Overview." eMedicine, August 11, 2006.
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