AL Activators

The designation AL in the context of proteins often refers to immunoglobulin light chains of the lambda type that are involved in the pathology of a condition known as AL amyloidosis. In AL amyloidosis, these light chains are produced in excess by abnormal plasma cells in the bone marrow and can misfold, forming amyloid fibrils that deposit in tissues, leading to organ dysfunction.Immunoglobulin light chains are a component of antibodies (immunoglobulins) that are crucial for the immune response. Antibodies are Y-shaped molecules consisting of two heavy chains and two light chains. The light chains come in two types: kappa (κ) and lambda (λ). Normally, these chains are produced in a controlled manner and are stable, soluble proteins that do not aggregate.

In the case of AL amyloidosis, the light chains (most commonly lambda type, hence "AL") are overproduced and have an abnormal structure that predisposes them to misfolding and forming insoluble fibrils. These fibrils accumulate in various tissues, including the heart, kidneys, liver, and nervous system, impairing their normal function. The deposition of amyloid fibrils in the tissues is a hallmark of amyloidosis, and AL amyloidosis is the most common type.Indicators of AL amyloidosis are varied and depend on the organs affected but can include fatigue, weight loss, swelling, numbness, and heart irregularities. Diagnosis is typically made through biopsies of affected tissues, where the amyloid deposits can be identified with specific staining techniques.