ArticlePloS one2007
Germ line origin and somatic mutations determine the target tissues in systemic AL-amyloidosis.
Article in PloS one, 2007. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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Who cites it
18 citing papers in PubMed, 48 citations in OpenAlex.
- Pathogenesis and kidney prognosis of renal amyloidosis.Cellular and molecular life sciences : CMLS · 2026Review
- An updated AL-base reveals ranked enrichment of immunoglobulin light chain variable genes in AL amyloidosis.Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis · 2025Article
- Kinetic evidence for multiple aggregation pathways in antibody light chain variable domains.Protein science : a publication of the Protein Society · 2024Article
- Atypical Presentation of Amyloidosis in a Female Patient with Muscle Weakness.Case reports in medicine · 2023Article
- Tissue-based diagnosis of systemic amyloidosis: Experience of the informal diagnostic center at Uppsala University Hospital.Upsala journal of medical sciences · 2022Article
- Dissecting the Molecular Features of Systemic Light Chain (AL) Amyloidosis: Contributions from Proteomics.Medicina (Kaunas, Lithuania) · 2021Review
- Genetic pathogenesis of immunoglobulin light chain amyloidosis: basic characteristics and clinical applications.Experimental hematology & oncology · 2021Review
- IGVL gene region usage correlates with distinct clinical presentation in IgM vs non-IgM light chain amyloidosis.Blood advances · 2021Article
- Article
- Different Dynamics in 6aJL2 Proteins Associated with AL Amyloidosis, a Conformational Disease.International journal of molecular sciences · 2019Article
- Article
- A Rare Case of Systemic AL Amyloidosis with Muscle Involvement: A Misleading Diagnosis.Case reports in hematology · 2018Article
- Systemic amyloidosis manifested by gastric outlet obstruction.Clinical endoscopy · 2013Article
- Somatic alpha-synuclein mutations in Parkinson's disease: hypothesis and preliminary data.Movement disorders : official journal of the Movement Disorder Society · 2013Article
- The pathogenesis and diagnosis of acute kidney injury in multiple myeloma.Nature reviews. Nephrology · 2011Review
- Utility of Doppler myocardial imaging, cardiac biomarkers, and clonal immunoglobulin genes to assess left ventricular performance and stratify risk following peripheral blood stem cell transplantation in patients with systemic light chain amyloidosis (Al).Journal of the American Society of Echocardiography : official publication of the American Society of Echocardiography · 2011Article
- Structural typing of systemic amyloidoses by luminescent-conjugated polymer spectroscopy.The American journal of pathology · 2010Article
- Molecular mechanism of amyloidogenic mutations in hypervariable regions of antibody light chains.The Journal of biological chemistryArticle
Corrections and comments
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Authors and funding
5 authors at 4 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundAmyloid is insoluble aggregated proteins deposited in the extra cellular space. About 25 different proteins are known to form amyloid in vivo and are associated with severe diseases such as Alzheimer's disease, prion diseases and type-2 diabetes. Light chain (AL) -amyloidosis is unique among amyloid diseases in that the fibril protein, a monoclonal immunoglobulin light chain, varies between individuals and that no two AL-proteins with identical primary structures have been described to date. The variability in tissue distribution of amyloid deposits is considerably larger in systemic AL-amyloidosis than in any other form of amyloidosis. The reason for this variation is believed to be based on the differences in properties of the amyloidogenic immunoglobulin light chain. However, there is presently no known relationship between the structure of an AL-protein and tissue distribution. METHODOLOGY/PRINCIPAL
findingsWe compared the pattern of amyloid deposition in four individuals with amyloid protein derived from variable light chain gene O18-O8, the source of a high proportion of amyloidogenic light chains, and in whom all or most of the fibril protein had been determined by amino acid sequencing. In spite of great similarities between the structures of the proteins, there was a pronounced variability in deposition pattern. We also compared the tissue distribution in these four individuals with that of four other patients with AL-amyloid derived from the L2-L16 gene. Although the interindividual variations were pronounced, liver and kidney involvement was much more evident in the latter four. CONCLUSIONS/SIGNIFICANCE: We conclude that although the use of a specific gene influences the tissue distribution of amyloid, each light chain exhibits one or more determinants of organ-specificity, which originate from somatic mutations and post-translational modifications. Eventual identification of such determinants could lead to improved treatment of patients with AL amyloidosis.
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