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Target intelligence / Profile preview
Misfolded Immunoglobulin Light Chain (LC)
- Target
- LC
- Molecular classification
- Immunoglobulin, Protein, Amyloid protein
01
Overview
Misfolded immunoglobulin light chains are abnormal protein fragments produced by clonal plasma cells, most notably in the context of AL (amyloid light-chain) amyloidosis. Normally, immunoglobulins (antibodies) are composed of two heavy and two light chains; the light chains can be either kappa (κ) or lambda (λ), but a given antibody contains only one type of light chain. In AL amyloidosis, excessive production and misfolding of these free light chains lead to their aggregation into insoluble amyloid fibrils that deposit in tissues and organs. Misfolded immunoglobulin light chain is both a biomarker for diagnosis/prognosis and a therapeutic target in AL amyloidosis. Treatments aim at reducing production/secretion from malignant plasma cells or directly targeting/removing deposited amyloid.
- Other names
- Free Light Chains (FLC)Amyloid Light Chainsκ light chainλ light chain
02
Mechanism of action
Reducing production/secretion from malignant plasma cells or directly targeting/removing deposited amyloid.
03
Biological functions
Immune response (when properly folded)Protein aggregationAmyloid fibril formationTissue deposition
04
Disease associations
AL AmyloidosisPlasma cell disordersKidney diseaseHeart diseaseLiver diseaseNeuropathy
05
Safety considerations
Multi-organ dysfunctionAmyloid fibril deposition in vital organsTreatment-related toxicities
06
Biomarkers
Monoclonal free κ or λ immunoglobulin light chainSerum free light chain assayUrine free light chain assay
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