Asset Details
Do you wish to reserve the book?
Molecular Mechanism of Pathogenesis and Treatment Strategies for AL Amyloidosis
Hey, we have placed the reservation for you!
By the way, why not check out events that you can attend while you pick your title.
Oops! Something went wrong.
Looks like we were not able to place the reservation. Kindly try again later.
Are you sure you want to remove the book from the shelf?
Molecular Mechanism of Pathogenesis and Treatment Strategies for AL Amyloidosis
Do you wish to request the book?
Molecular Mechanism of Pathogenesis and Treatment Strategies for AL Amyloidosis
Please be aware that the book you have requested cannot be checked out. If you would like to checkout this book, you can reserve another copy
We have requested the book for you!
Your request is successful and it will be processed during the Library working hours. Please check the status of your request in My Requests.
Oops! Something went wrong.
Looks like we were not able to place your request. Kindly try again later.
Journal Article
Molecular Mechanism of Pathogenesis and Treatment Strategies for AL Amyloidosis
2022
Overview
In amyloid light-chain (AL) amyloidosis, small B-cell clones (mostly plasma cell clones) present in the bone marrow proliferate and secrete unstable monoclonal free light chains (FLCs), which form amyloid fibrils that deposit in the interstitial tissue, resulting in organ injury and dysfunction. AL amyloidosis progresses much faster than other types of amyloidosis, with a slight delay in diagnosis leading to a marked exacerbation of cardiomyopathy. In some cases, the resulting heart failure is so severe that chemotherapy cannot be administered, and death sometimes occurs within a few months. To date, many clinical studies have focused on therapeutics, especially chemotherapy, to treat this disease. Because it is necessary to promptly lower FLC, the causative protein of amyloid, to achieve a hematological response, various anticancer agents targeting neoplastic plasma cells are used for the treatment of this disease. In addition, many basic studies using human specimens to elucidate the pathophysiology of AL have been conducted. Gene mutations associated with AL, the characteristics of amyloidogenic LC, and the structural specificity of amyloid fibrils have been clarified. Regarding the mechanism of cellular and tissue damage, the mass effect due to amyloid deposition, as well as the toxicity of pre-fibrillar LC, is gradually being elucidated. This review outlines the pathogenesis and treatment strategies for AL amyloidosis with respect to its molecular mechanisms.
Publisher
MDPI AG,MDPI
Subject
/ Amyloid
/ Disease
/ Humans
/ Immunoglobulin Light Chains - chemistry
/ Immunoglobulin Light-chain Amyloidosis
/ Immunoglobulin Light-chain Amyloidosis - diagnosis
/ Immunoglobulin Light-chain Amyloidosis - genetics
/ Immunoglobulin Light-chain Amyloidosis - therapy
/ Mutation
/ Plasma
/ Proteins
/ Review
