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©2013 UpToDate ® Print Email Protein precursor of amyloid deposits-I Protein class Precursor protein (abbreviation) Amyloid type Clinical type High density apolipoproteins (Apo) serum AA AA Associated with amyloid complicating chronic infections or inflammatory diseases, and some heredofamilial periodic fever syndromes, such as FMF Apolipoprotein A-I (ApoAI) AApoAI Age-related amyloid occurring in the aortic intima, and some hereditary neuropathic or cardiopathic amyloidoses [1,2] Apolipoprotein A-II (ApoAII) AApoAII Some hereditary nephropathic amyloidoses [3] Immunoglobulin (Ig) gene superfamily Ig L chain/Ig H chains (IgL/IgH) AL/AH Primary and myeloma-associated amyloidosis Beta-2 microglobulin Aβ2m Dialysis amyloidosis Neuroendocrine (Pro)Calcitonin ACal Amyloid complicating C-cell thyroid tumors Islet amyloid AIAPP Islet cell amyloid in Insulinomas, type II diabetes mellitus, and aging [4] Atrial natriuretic peptide AANF Isolated atrial amyloidosis of aging Prolactin/Apro APro Prolactinomas/aging Insulin Alns Local amyloid complicating use of the insulin pump Cytoskeleton-related Gelsolin AGel Hereditary neuropathic amyloid associated with corneal lattice dystrophy and cutis laxa (Meretoja syndrome) [5] Keratin * Cutaneous amyloid Keratoepithelin AKer Hereditary granular, lattice, and avellino corneal dystrophies [6] * Does not yet have nomenclature designated. References: Mucchiano GI, Haggqvist B, Sletten K, Westermark P, J Pathol 2001; 193:270. de Sousa MM, Vital C, Ostler D, et al, Am J Pathol 2000; 156:1911. Benson MD, Liepnieks JJ, Yazaki M, et al, Genomics 2001; 72:272. Hoppener JW, Ahren B, Lips CJ. N Engl J Med 2000; 343:411. Kiuru S, Amyloid 1998; 5:55. Klintworth GK. Front Biosci 2003; 8:d687.

©2013 UpToDate ® Print Email Protein precursor of amyloid deposits-I Protein class Precursor protein (abbreviation) Amyloid type Clinical type High density apolipoproteins (Apo) serum AA AA Associated with amyloid complicating chronic infections or inflammatory diseases, and some heredofamilial periodic fever syndromes, such as FMF Apolipoprotein A-I (ApoAI) AApoAI Age-related amyloid occurring in the aortic intima, and some hereditary neuropathic or cardiopathic amyloidoses [1,2] Apolipoprotein A-II (ApoAII) AApoAII Some hereditary nephropathic amyloidoses [3] Immunoglobulin (Ig) gene superfamily Ig L chain/Ig H chains (IgL/IgH) AL/AH Primary and myeloma-associated amyloidosis Beta-2 microglobulin Aβ2m Dialysis amyloidosis Neuroendocrine (Pro)Calcitonin ACal Amyloid complicating C-cell thyroid tumors Islet amyloid AIAPP Islet cell amyloid in Insulinomas, type II diabetes mellitus, and aging [4] Atrial natriuretic peptide AANF Isolated atrial amyloidosis of aging Prolactin/Apro APro Prolactinomas/aging Insulin Alns Local amyloid complicating use of the insulin pump Cytoskeleton-related Gelsolin AGel Hereditary neuropathic amyloid associated with corneal lattice dystrophy and cutis laxa (Meretoja syndrome) [5] Keratin * Cutaneous amyloid Keratoepithelin AKer Hereditary granular, lattice, and avellino corneal dystrophies [6] * Does not yet have nomenclature designated. References: Mucchiano GI, Haggqvist B, Sletten K, Westermark P, J Pathol 2001; 193:270. de Sousa MM, Vital C, Ostler D, et al, Am J Pathol 2000; 156:1911. Benson MD, Liepnieks JJ, Yazaki M, et al, Genomics 2001; 72:272. Hoppener JW, Ahren B, Lips CJ. N Engl J Med 2000; 343:411. Kiuru S, Amyloid 1998; 5:55. Klintworth GK. Front Biosci 2003; 8:d687. Protein precursor of amyloid deposits-II Protein class Precursor protein (abbreviation) Amyloid type Clinical type Transport protein Transthyretin (TTR; prealbumin) ATTR Hereditary neuropathic and/or cardiopathic amyloids; vitreous amyloidosis; leptomeningeal or renal amyloid in some kindreds; senile systemic amyloidosis [1] Cerebrovascular/ neurdegeneration Amyloid precursor protein (APP) ABeta Hereditary and sporadic Alzheimer disease; congophilic cerebral angiopathy [2,3] Prion protein (PRP) APrPsc Hereditary and sporadic spongiform encephalopathies [4] BRI gene product ABri/ADan Hereditary dementias (British and Danish types) [5] Cystatin C (Cys - C) ACys Hereditary cerebrovascular hemorrhage with amyloidosis (Icelandic type) [6] Coagulation protein Fibrinogen alpha chain AFib Hereditary nephropathic amyloidosis [7] Enzyme Lysozome

Prion protein (PRP) APrPsc Hereditary and sporadic spongiform encephalopathies [4] BRI gene product ABri/ADan Hereditary dementias (British and Danish types) [5] Cystatin C (Cys - C) ACys Hereditary cerebrovascular hemorrhage with amyloidosis (Icelandic type) [6] Coagulation protein Fibrinogen alpha chain AFib Hereditary nephropathic amyloidosis [7] Enzyme Lysozome ALys Hereditary nephropathic amyloidosis; may have marked hepatic, splenic and gastrointestinal amyloid deposits [7] Other Keratoepithelin AKer Various familial corneal dystrophies [8] Lactoferrin ALac Corneal amyloidosis associated with trichiasis Odontogenic ameloblast-associated protein AOaap Calcifying epithelial odontogenic tumors (CEOTs) Semenogelin 1 ASem1 Senile seminal vesicle amyloid Lactadherin AMed Senile aortic amyloid; media deposition Leukocyte chemotactic factor 2 ALect2 Amyloid nephropathy References: Connors LH, Lom A, Prokaeva T, et al. Amyloid 2003; 10:160 (TTR variants updated at file://medicine.bu.edu/amyloid/Amyloidl.htm). Selkoe DJ. Physiol Revs 2001; 81:741. Revesz T, Ghiso J, Lashley T, et al. J Neuropathol Exp Neurol 2003; 62:885. Prusiner SB. N Engl J Med 2001; 344:1516. Vidal R, Frangione B, Rostagno A, et al. Nature 1999; 399:776 and Gibson G, Gunasekera N, Lee M, et al. J Neurochem 2004; 88:281. Olafsson I, Grubb A. Amyloid 2000; 7:70. Hawkins PN. J Nephrol 2003; 16:443. Klintworth GK. Front Biosci 2003; 8:d687. Sipe JD, Benson MD, Buxbaum JN, et al. Amyloid 2010; 17:101.