Merrill D Benson, Indiana University School of Medicine, Indianapolis, Indiana, USA
Published online: July 2008
DOI: 10.1002/9780470015902.a0002146.pub2
The amyloidoses are a group of -structure protein deposition diseases that may be systemic or localized, sporadic, hereditary or associated with chronic inflammation. Pathology is the result of displacement of normal tissue structures.
Keywords: amyloid; amyloidosis; protein deposition diseases
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Figure 1. Histological sections of an endomyocardial biopsy from a patient with amyloid cardiomyopathy. (a) Amyloid deposits are eosinophilic and disrupt normal myocardial fibres (haemotoxylin and eosin). (b) Amyloid deposits have an affinity for Congo red. (c) Same section as in (b) in polarized light showing green birefringence characteristic of Congo red-stained amyloid fibril deposits.
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Figure 2. Computer graphic model of a I amyloid light chain variable region protein (V
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Figure 3. Computer graphic model of transthyretin tetramer. Thyroxine binds in the central channel and vitamin A–RBP binds on the surface of the tetramer. Each monomer has extensive sheet structure.
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| References | |
| book Benson MD (2003) "The hereditary amyloidoses". In: Lioté F and Pascual E (eds) Best Practice and Research Clinical Rheumatology, vol. 17, chap. 2, pp. 909–927 Amsterdam: Elsevier. | |
| Benson MD and Kincaid JC (2007) Invited review: the molecular biology and clinical features of amyloid neuropathy. Muscle & Nerve 36: 411–423. | |
| Glenner GG (1980) Amyloid deposits and amyloidosis: the -fibrilloses. New England Journal of Medicine 302: 1283–1292, 1333–1343. | |
| Lachman HJ, Booth DR, Booth SE et al. (2002) Misdiagnosis of hereditary amyloidosis as AL (primary) amyloidosis. New England Journal of Medicine 346: 1786–1791. | |
| Levy E, Carman MD, Fernandez-Madrid IJ et al. (1990) Mutation of the Alzheimer's disease amyloid gene in hereditary cerebral hemorrhage, Dutch type. Science 248: 1124–1126. | |
| Schormann N, Murrell JR, Liepnieks JJ and Benson MD (1995) Tertiary structure of an amyloid immunoglobulin light chain protein: a proposed model for amyloid fibril formation. Proceedings of the National Academy of Sciences of the USA 92: 9490–9494. | |
| Skinner M, Sanchorawala V, Seldin DC et al. (2004) High-dose Melphalan and autologous stem-cell transplantation in patients with AL amyloidosis: an 8-year study. Annals of Internal Medicine 140: 85–93. | |
| Westermark P, Benson MD, Buxbaum JN et al. (2007) A primer of amyloid nomenclature. Amyloid 14: 179–183. | |
| Further Reading | |
| book Benson MD (2001) "Amyloidosis". In: Scriver CR, Beaudet AL, Sly WS and Valle D (eds) The Metabolic and Molecular Bases of Inherited Disease, 8th edn, part 22, Connective Tissues, vol. IV, chap. 209, pp. 5345–5378. New York: McGraw-Hill. | |
| book Benson MD (2005a) "Amyloidosis". In: Koopman WJ and Moreland LW (eds) Arthritis and Allied Conditions: A Textbook of Rheumatology, 15th edn, vol. 2, chap. 93, pp. 1933–1960. Philadelphia: Williams & Wilkins, A Waverly Company. | |
| book Benson MD (2005b) "Amyloidosis and other protein deposition diseases". In: Rimoin DL, Connor JM, Pyeritz RE, and Korf BR Emery and Rimoin's (eds) Principles and Practice of Medical Genetics, 5th edn, vol. 2, chap. 83, pp. 1821–1834. Philadelphia: Churchill Livingstone publishers. | |
| Benson MD and Uemichi T (1996) Review: transthyretin amyloidosis. Amyloid. International Journal of Experimental and Clinical Investigation 3: 44–56. | |
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