Immunoglobulin light chain amyloid aggregation
نویسندگان
چکیده
منابع مشابه
An Immunoglobulin Light Chain
Identification of the immunogens responsible for the production of pathogenic autoantibodies is a major problem in the field of autoimmunity. In the principal diseases that are thought to involve autoimmunization, rheumatoid arthritis, type I diabetes, and multiple sclerosis are examples, the instigating stimulus is unknown. Another example of the gap in our knowledge of disease-causing immunog...
متن کاملTertiary structure of an amyloid immunoglobulin light chain protein: a proposed model for amyloid fibril formation.
An immunoglobulin light chain protein was isolated from the urine of an individual (BRE) with systemic amyloidosis. Complete amino acid sequence of the variable region of the light chain (VL) protein established it as a kappa I, which when compared with other kappa I amyloid associated proteins had unique residues, including Ile-34, Leu-40, and Tyr-71. To study the tertiary structure, BRE VL wa...
متن کاملAtypical immunoglobulin light chain amyloidosis
BACKGROUND Primary immunoglobulin light chain amyloidosis (AL amyloidosis) is a plasma cell disorder which mainly affects heart, kidneys, liver, and peripheral nervous system. Cases of atypical AL amyloidosis presented as spontaneous vertebral compression fractures have been rarely reported, and data about the management and clinical outcomes of the patients are scarce. METHODS Herein, we pre...
متن کاملFunctional Immunoglobulin Light Chain Genes
Rearrangement of Ig genes proceeds during B cell differentiation in an orderly fashion. First the heavy (H) chain gene is rearranged in pre-B cells . These cells can express cytoplasmic A chain but no light (L) chain (1). The K light chain genes rear range next and if they fail to produce a functional gene on either chromosome the X light chain genes are rearranged (2) . It is generally believe...
متن کاملThe amyloid fibrils of the constant domain of immunoglobulin light chain.
Light chain-associated (AL) amyloidosis is characterized by dominant fibril deposition of the variable domain (VL) of an immunoglobulin light chain, and thus its constant domain (CL) has been considered not to be amyloidogenic. We examined the in vitro fibril formation of the isolated CL in comparison with beta2-microglobulin (beta2-m), an immunoglobulin domain-like amyloidogenic protein respon...
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ژورنال
عنوان ژورنال: Chemical Communications
سال: 2018
ISSN: 1359-7345,1364-548X
DOI: 10.1039/c8cc04396e