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  1. Article ; Online: Inotersen treatment for ATTR amyloidosis.

    Benson, Merrill D

    Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis

    2019  Volume 26, Issue sup1, Page(s) 27–28

    MeSH term(s) Amyloid Neuropathies, Familial/drug therapy ; Amyloid Neuropathies, Familial/genetics ; Amyloid Neuropathies, Familial/pathology ; Drug-Related Side Effects and Adverse Reactions/classification ; Drug-Related Side Effects and Adverse Reactions/pathology ; Female ; Humans ; Male ; Oligonucleotides/administration & dosage ; Oligonucleotides/adverse effects ; Oligonucleotides, Antisense/administration & dosage ; Oligonucleotides, Antisense/genetics ; Prealbumin/genetics
    Chemical Substances Oligonucleotides ; Oligonucleotides, Antisense ; Prealbumin ; Inotersen (0IEO0F56LV)
    Language English
    Publishing date 2019-08-09
    Publishing country England
    Document type Clinical Trial, Phase III ; Journal Article ; Randomized Controlled Trial
    ZDB-ID 1205246-2
    ISSN 1744-2818 ; 1350-6129
    ISSN (online) 1744-2818
    ISSN 1350-6129
    DOI 10.1080/13506129.2019.1582497
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    Zs.A 4114: Show issues Location:
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  2. Article ; Online: Editorial Commentary: Advances in transthyretin amyloid cardiomyopathy.

    Benson, Merrill D / Dasgupta, Noel R

    Trends in cardiovascular medicine

    2020  Volume 31, Issue 1, Page(s) 67–68

    MeSH term(s) Amyloid Neuropathies, Familial/diagnosis ; Amyloid Neuropathies, Familial/genetics ; Amyloid Neuropathies, Familial/therapy ; Cardiomyopathies/diagnosis ; Cardiomyopathies/genetics ; Cardiomyopathies/therapy ; Heart Failure ; Humans ; Prealbumin/genetics ; Stroke Volume
    Chemical Substances Prealbumin
    Language English
    Publishing date 2020-01-07
    Publishing country United States
    Document type Editorial ; Comment
    ZDB-ID 1097434-9
    ISSN 1873-2615 ; 1050-1738
    ISSN (online) 1873-2615
    ISSN 1050-1738
    DOI 10.1016/j.tcm.2019.12.008
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    Zs.A 3419: Show issues Location:
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  3. Article ; Online: Transthyretin amyloidosis: a little history of hereditary amyloidosis.

    Benson, Merrill D

    Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis

    2017  Volume 24, Issue sup1, Page(s) 76–77

    MeSH term(s) Amyloid Neuropathies, Familial/genetics ; Amyloid Neuropathies, Familial/history ; Amyloid Neuropathies, Familial/pathology ; Amyloid Neuropathies, Familial/therapy ; Apolipoproteins/genetics ; Apolipoproteins/metabolism ; Doxycycline/therapeutic use ; Female ; Gene Expression Regulation ; History, 20th Century ; History, 21st Century ; Humans ; Male ; Myocardium/metabolism ; Myocardium/pathology ; Nervous System/metabolism ; Nervous System/pathology ; Oligonucleotides, Antisense/genetics ; Oligonucleotides, Antisense/metabolism ; Prealbumin/antagonists & inhibitors ; Prealbumin/genetics ; Prealbumin/history ; Prealbumin/metabolism ; Protein Multimerization ; RNA, Messenger/antagonists & inhibitors ; RNA, Messenger/genetics ; RNA, Messenger/metabolism ; RNA, Small Interfering/genetics ; RNA, Small Interfering/metabolism
    Chemical Substances Apolipoproteins ; Oligonucleotides, Antisense ; Prealbumin ; RNA, Messenger ; RNA, Small Interfering ; Doxycycline (N12000U13O)
    Language English
    Publishing date 2017-05-01
    Publishing country England
    Document type Historical Article ; Letter
    ZDB-ID 1205246-2
    ISSN 1744-2818 ; 1350-6129
    ISSN (online) 1744-2818
    ISSN 1350-6129
    DOI 10.1080/13506129.2017.1281116
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: Treatment of ATTR cardiomyopathy with a TTR specific antisense oligonucleotide, inotersen.

    Dasgupta, Noel R / Benson, Merrill D

    Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis

    2019  Volume 26, Issue sup1, Page(s) 20–21

    MeSH term(s) Aged ; Amyloid Neuropathies, Familial/drug therapy ; Amyloid Neuropathies, Familial/genetics ; Amyloid Neuropathies, Familial/pathology ; Cardiomyopathies/drug therapy ; Cardiomyopathies/genetics ; Cardiomyopathies/pathology ; Female ; Humans ; Male ; Middle Aged ; Oligonucleotides/administration & dosage ; Oligonucleotides, Antisense/administration & dosage ; Prealbumin/genetics
    Chemical Substances Oligonucleotides ; Oligonucleotides, Antisense ; Prealbumin ; TTR protein, human ; Inotersen (0IEO0F56LV)
    Language English
    Publishing date 2019-08-09
    Publishing country England
    Document type Clinical Study ; Journal Article
    ZDB-ID 1205246-2
    ISSN 1744-2818 ; 1350-6129
    ISSN (online) 1744-2818
    ISSN 1350-6129
    DOI 10.1080/13506129.2019.1582490
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: Post-translational modification of amyloid a protein in patients with AA amyloidosis.

    Kluve-Beckerman, Barbara / Smith, Justin T / Ivancic, Carlie / Benson, Merrill D

    Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis

    2021  Volume 29, Issue 1, Page(s) 50–57

    Abstract: AA amyloidosis is a disease caused by extracellular deposition of insoluble β-pleated sheet fibrils composed of amyloid A (AA) protein, an amino (N)-terminal fragment of serum amyloid A (SAA). The deposits disrupt tissue structure and compromise organ ... ...

    Abstract AA amyloidosis is a disease caused by extracellular deposition of insoluble β-pleated sheet fibrils composed of amyloid A (AA) protein, an amino (N)-terminal fragment of serum amyloid A (SAA). The deposits disrupt tissue structure and compromise organ function. Although the disease is systemic, deposition in kidney glomeruli is the most common manifestation. The leading cause of AA amyloidosis is sustained or recurrent inflammation accompanied by elevated levels of SAA. Factors determining the conversion of SAA to AA amyloid fibrils have yet to be fully resolved. Herein, we present liquid chromatography tandem-mass spectrometry (LC-MS/MS) analysis of AA proteins purified from eight patients with AA amyloidosis. For the first time, post-translational modifications (PTM), including carbamylation, acetylation and oxidation, were identified on AA peptides; all eight samples showed some degree of PTM. The amyloid in 6 samples comprised peptides derived from SAA1 with few or none from SAA2, while the other two samples contained both SAA1- and SAA2-derived peptides. N-terminal AA peptides beginning with Arg1 as well as AA peptides starting with Ser2 were present in five of the eight samples, while all or nearly all of the N-terminal peptides in the other three samples lacked Arg1. These data demonstrate that multiple species of AA amyloid proteins can comprise the subunits in amyloid fibrils and raise the possibility that PTM may play a role in fibrillogenesis.
    MeSH term(s) Amyloid/metabolism ; Amyloidosis ; Chromatography, Liquid ; Humans ; Protein Processing, Post-Translational ; Serum Amyloid A Protein/metabolism ; Tandem Mass Spectrometry
    Chemical Substances Amyloid ; Serum Amyloid A Protein
    Language English
    Publishing date 2021-11-17
    Publishing country England
    Document type Journal Article
    ZDB-ID 1205246-2
    ISSN 1744-2818 ; 1350-6129
    ISSN (online) 1744-2818
    ISSN 1350-6129
    DOI 10.1080/13506129.2021.1997985
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: ALECT2 and NASH.

    Cummings, Oscar W / Dasgupta, Noel R / Benson, Merrill D

    Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis

    2019  Volume 26, Issue sup1, Page(s) 158–159

    MeSH term(s) Amyloidosis/diagnosis ; Amyloidosis/metabolism ; Amyloidosis/pathology ; Female ; Humans ; Intercellular Signaling Peptides and Proteins/metabolism ; Liver/metabolism ; Liver/pathology ; Liver Cirrhosis/diagnosis ; Liver Cirrhosis/metabolism ; Liver Cirrhosis/pathology ; Middle Aged
    Chemical Substances Intercellular Signaling Peptides and Proteins ; LECT2 protein, human
    Language English
    Publishing date 2019-07-25
    Publishing country England
    Document type Case Reports ; Journal Article
    ZDB-ID 1205246-2
    ISSN 1744-2818 ; 1350-6129
    ISSN (online) 1744-2818
    ISSN 1350-6129
    DOI 10.1080/13506129.2019.1584099
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article ; Online: Liver transplantation and transthyretin amyloidosis.

    Benson, Merrill D

    Muscle & nerve

    2013  Volume 47, Issue 2, Page(s) 157–162

    Abstract: Liver transplantation as a specific treatment of transthyretin amyloidosis was first performed in 1990. The rationale for this treatment was that removal of the source (liver) of the amyloid precursor protein (mutated transthyretin) would stop ... ...

    Abstract Liver transplantation as a specific treatment of transthyretin amyloidosis was first performed in 1990. The rationale for this treatment was that removal of the source (liver) of the amyloid precursor protein (mutated transthyretin) would stop progression of the disease. Indeed, after orthotopic liver transplantation (OLT), mutant transthyretin (TTR) is rapidly cleared from circulation. In the last 20 years, >2000 familial amyloidotic polyneuropathy (FAP) patients have received liver transplants. For these patients, prospective monitoring has shown prolongation of life compared with FAP patients who have not undergone liver transplantation. The most favorable results have been for FAP patients with the Val30Met TTR mutation. Less favorable results have been seen for patients with other TTR mutations where progression of amyloid tissue deposition has been documented as the result of amyloid fibril formation from normal (wild-type) TTR. Although it is obvious that OLT has benefited many FAP patients, there remains a need for further therapies.
    MeSH term(s) Amyloid Neuropathies, Familial/surgery ; Disease Progression ; Humans ; Liver Transplantation ; Treatment Outcome
    Language English
    Publishing date 2013-02
    Publishing country United States
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't ; Research Support, U.S. Gov't, Non-P.H.S. ; Review
    ZDB-ID 438353-9
    ISSN 1097-4598 ; 0148-639X
    ISSN (online) 1097-4598
    ISSN 0148-639X
    DOI 10.1002/mus.23521
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    Zs.A 1449: Show issues Location:
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    Zs.MO 551: Show issues

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  8. Article ; Online: Amyloid Cardiomyopathy.

    Benson, Merrill D / Dasgupta, Noel R

    Journal of the American College of Cardiology

    2016  Volume 68, Issue 1, Page(s) 25–28

    MeSH term(s) Amyloid ; Amyloidosis ; Biopsy ; Cardiomyopathies ; Humans ; Prealbumin
    Chemical Substances Amyloid ; Prealbumin
    Language English
    Publishing date 2016-07-01
    Publishing country United States
    Document type Editorial ; Comment
    ZDB-ID 605507-2
    ISSN 1558-3597 ; 0735-1097
    ISSN (online) 1558-3597
    ISSN 0735-1097
    DOI 10.1016/j.jacc.2016.05.009
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    Zs.A 1846: Show issues Location:
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  9. Article ; Online: Pathogenesis of transthyretin amyloidosis.

    Benson, Merrill D

    Amyloid : the international journal of experimental and clinical investigation : the official journal of the International Society of Amyloidosis

    2012  Volume 19 Suppl 1, Page(s) 14–15

    Abstract: Current dogma for transthyretin (TTR) pathogenesis is that mutations in TTR alter its structure such that the tetramer becomes unstable and prone to release of monomer which then becomes the putative building block of the fibril. This hypothesis is ... ...

    Abstract Current dogma for transthyretin (TTR) pathogenesis is that mutations in TTR alter its structure such that the tetramer becomes unstable and prone to release of monomer which then becomes the putative building block of the fibril. This hypothesis is supported by thermodynamic data showing decreased stability of mutant TTR tetrameric proteins and accelerated fibril formation under acidic conditions in vitro. There are, however, a number of questions that are not readily answered by this simplistic model of a very complex disease. Worrisome questions still to be answered include: 1. If the monomer is the precursor of the fibril, why do fibril deposits contain large amounts of wild-type TTR and not just variant? 2. If destabilized tetramers can form fibrils in vitro, why do we consistently find partial proteolysis of fibril subunit proteins? If enzymatic proteolysis is a required step in fibril formation, are the findings of in vitro fibril formation relevant to the true pathogenesis? 3. With some TTR mutations (e.g. 122ΔVal), it would appear that very little TTR is present in the blood (probably due to degradation prior to hepatic secretion). Enough mutant TTR circulates to the heart and nerves to cause pathology but, if the mutant only serves to initiate fibril deposition, why are not the deposits mainly wild-type TTR?4. Since mutated TTR is present from birth, why is TTR amyloidosis of such delayed onset? What is the role of aging factors?5. Do the variations in biochemical analyses of heart and nerve versus choroid and leptomeningeal fibrils tell us something about pathogenesis? These are questions we need to address. Do not expect quick and easy answers. Hopefully, they will generate thought and discussion.
    MeSH term(s) Amyloidosis/genetics ; Amyloidosis/metabolism ; Amyloidosis/pathology ; Humans ; Prealbumin/genetics ; Prealbumin/metabolism ; Protein Multimerization
    Chemical Substances Prealbumin
    Language English
    Publishing date 2012-06
    Publishing country England
    Document type Journal Article
    ZDB-ID 1205246-2
    ISSN 1744-2818 ; 1350-6129
    ISSN (online) 1744-2818
    ISSN 1350-6129
    DOI 10.3109/13506129.2012.668501
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    Zs.A 4114: Show issues Location:
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  10. Article ; Online: LECT2 amyloidosis.

    Benson, Merrill D

    Kidney international

    2010  Volume 77, Issue 9, Page(s) 757–759

    Abstract: LECT2 amyloidosis is the latest systemic type of amyloidosis to be described. It was discovered in patients with nephrotic syndrome and renal failure and is characterized by amyloid deposition in glomeruli, renal vessels, and interstitium. Clinical and ... ...

    Abstract LECT2 amyloidosis is the latest systemic type of amyloidosis to be described. It was discovered in patients with nephrotic syndrome and renal failure and is characterized by amyloid deposition in glomeruli, renal vessels, and interstitium. Clinical and pathological features of earlier phases of this type of amyloidosis have yet to be determined.
    MeSH term(s) Amyloidosis/drug therapy ; Amyloidosis/pathology ; Humans ; Kidney/pathology ; Kidney Glomerulus/pathology ; Nephrotic Syndrome/pathology ; Renal Insufficiency/pathology
    Language English
    Publishing date 2010-05
    Publishing country United States
    Document type Comment ; Journal Article
    ZDB-ID 120573-0
    ISSN 1523-1755 ; 0085-2538
    ISSN (online) 1523-1755
    ISSN 0085-2538
    DOI 10.1038/ki.2010.18
    Database MEDical Literature Analysis and Retrieval System OnLINE

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