Title |
AL Amyloidosis
|
---|---|
Published in |
Orphanet Journal of Rare Diseases, August 2012
|
DOI | 10.1186/1750-1172-7-54 |
Pubmed ID | |
Authors |
Estelle Desport, Frank Bridoux, Christophe Sirac, Sébastien Delbes, Sébastien Bender, Béatrice Fernandez, Nathalie Quellard, Corinne Lacombe, Jean-Michel Goujon, David Lavergne, Julie Abraham, Guy Touchard, Jean-Paul Fermand, Arnaud Jaccard, Centre national de référence pour l’amylose AL et les autres maladies par dépôts d’immunoglobulines monoclonales |
Abstract |
DEFINITION OF THE DISEASE: AL amyloidosis results from extra-cellular deposition of fibril-forming monoclonal immunoglobulin (Ig) light chains (LC) (most commonly of lambda isotype) usually secreted by a small plasma cell clone. Most patients have evidence of isolated monoclonal gammopathy or smoldering myeloma, and the occurrence of AL amyloidosis in patients with symptomatic multiple myeloma or other B-cell lymphoproliferative disorders is unusual. The key event in the development of AL amyloidosis is the change in the secondary or tertiary structure of an abnormal monoclonal LC, which results in instable conformation. This conformational change is responsible for abnormal folding of the LC, rich in β leaves, which assemble into monomers that stack together to form amyloid fibrils. |
X Demographics
Geographical breakdown
Country | Count | As % |
---|---|---|
United States | 2 | 40% |
Spain | 1 | 20% |
United Kingdom | 1 | 20% |
Unknown | 1 | 20% |
Demographic breakdown
Type | Count | As % |
---|---|---|
Members of the public | 4 | 80% |
Scientists | 1 | 20% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
---|---|---|
Japan | 2 | 1% |
Spain | 2 | 1% |
Italy | 1 | <1% |
Unknown | 194 | 97% |
Demographic breakdown
Readers by professional status | Count | As % |
---|---|---|
Student > Bachelor | 32 | 16% |
Other | 29 | 15% |
Student > Ph. D. Student | 23 | 12% |
Researcher | 21 | 11% |
Student > Postgraduate | 18 | 9% |
Other | 43 | 22% |
Unknown | 33 | 17% |
Readers by discipline | Count | As % |
---|---|---|
Medicine and Dentistry | 95 | 48% |
Agricultural and Biological Sciences | 23 | 12% |
Biochemistry, Genetics and Molecular Biology | 17 | 9% |
Nursing and Health Professions | 5 | 3% |
Pharmacology, Toxicology and Pharmaceutical Science | 3 | 2% |
Other | 18 | 9% |
Unknown | 38 | 19% |