Evidence map›Paper›PMID 35683015›Full record

ReviewInternational journal of molecular sciences2022

Molecular Mechanism of Pathogenesis and Treatment Strategies for AL Amyloidosis.

Hidehiko Ikura, Jin Endo, Hiroki Kitakata, Hidenori Moriyama, Motoaki Sano, Keiichi Fukuda

Open access · goldAbstract readReview
In one paragraph

Review in International journal of molecular sciences, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
18citing papers in PubMed, 1 pooled it
2.6field-weighted citation impact, top 9% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

18 citing papers in PubMed, 1 synthesis or guideline pooled it, 33 citations in OpenAlex.

  1. Pooled it
  2. Review
  3. Article
  4. Article
  5. Review
  6. Biopsy-resolved cryo-EM structures of amyloid fibrils provide molecular insights into AL amyloidosis.Proceedings of the National Academy of Sciences of the United States of America · 2026
    Article
  7. Article
  8. Review
  9. Cardiac Amyloidosis: Tribulations and New Frontiers.Journal of personalized medicine · 2025
    Review
  10. Review
  11. Article
  12. Review
  13. Review
  14. Review
  15. Review
  16. Article
  17. Molecular Research on Amyloidosis.International journal of molecular sciences · 2023
    Article
  18. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

6 authors at 1 institution in 1 country.

Hidehiko IkuraDepartment of Cardiology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-City, Tokyo 160-8582, Japan.
Jin EndoDepartment of Cardiology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-City, Tokyo 160-8582, Japan.ORCID 0000-0001-7770-2519
Hiroki KitakataDepartment of Cardiology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-City, Tokyo 160-8582, Japan.
Hidenori MoriyamaDepartment of Cardiology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-City, Tokyo 160-8582, Japan.
Motoaki SanoDepartment of Cardiology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-City, Tokyo 160-8582, Japan.
Keiichi FukudaDepartment of Cardiology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-City, Tokyo 160-8582, Japan.
Keio University Hospital · JP

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

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.

Indexed as

AmyloidosisImmunoglobulin Light-chain AmyloidosisAmyloidAmyloidogenic ProteinsHumansImmunoglobulin Light ChainsAmyloidAmyloidogenic ProteinsImmunoglobulin Light Chainsamyloid light-chain (AL) amyloidosischemotherapymisfolded proteinsmolecular mechanismmonoclonal free light chains (FLCs)

Identifiers

PMID35683015
PMCPMC9181426
OpenAlexW4282052799

What OpenQuestion holds

Textmetadata
LicenceCC BY
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.