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  1. Book ; Online ; E-Book: Alpha-1-antitrypsin deficiency

    Strnad, Pavel / Brantly, Mark L. / Bals, Robert

    (ERS monograph)

    2019  

    Author's details edited by Pavel Strnad, Mark L. Brantly and Robert Bals
    Series title ERS monograph
    Keywords Electronic books
    Language English
    Size 1 Online-Ressource (xi, 195 Seiten), Illustrationen, Diagramme
    Publisher European Respiratory Society
    Publishing place Sheffield
    Publishing country Great Britain
    Document type Book ; Online ; E-Book
    Remark Zugriff für angemeldete ZB MED-Nutzerinnen und -Nutzer
    HBZ-ID HT020275019
    ISBN 978-1-84984-109-2 ; 9781849841085 ; 1-84984-109-8 ; 184984108X
    Database ZB MED Catalogue: Medicine, Health, Nutrition, Environment, Agriculture

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  2. Article ; Online: RNAi therapeutics for diseases involving protein aggregation: fazirsiran for alpha-1 antitrypsin deficiency-associated liver disease.

    Strnad, Pavel / San Martin, Javier

    Expert opinion on investigational drugs

    2023  Volume 32, Issue 7, Page(s) 571–581

    Abstract: Introduction: Therapeutic agents that prevent protein misfolding or promote protein clearance are being studied to treat proteotoxic diseases. Among them, alpha-1 antitrypsin deficiency (AATD) is caused by mutations in the alpha-1 antitrypsin (: Areas ...

    Abstract Introduction: Therapeutic agents that prevent protein misfolding or promote protein clearance are being studied to treat proteotoxic diseases. Among them, alpha-1 antitrypsin deficiency (AATD) is caused by mutations in the alpha-1 antitrypsin (
    Areas covered: This article describes the role of misfolded proteins and protein aggregates in disease and options for therapeutic approaches. The RNAi mechanism is discussed, along with how the siRNA therapeutic fazirsiran for the treatment of AATD was developed. We also describe the implications of siRNA therapeutics in extrahepatic diseases.
    Expert opinion: Using RNAi as a therapeutic approach is well suited to treat disease in conditions where an excess of a protein or the effect of an abnormal mutated protein causes disease. The results observed for the first few siRNA therapeutics that were approved or are in development provide an important promise for the development of future drugs that can address such conditions in a specific and targeted way. Current developments should enable the use of RNAi therapeutics outside the liver, where there are many more possible diseases to address.
    MeSH term(s) Humans ; Protein Aggregates ; RNA Interference ; RNAi Therapeutics ; alpha 1-Antitrypsin Deficiency/complications ; alpha 1-Antitrypsin Deficiency/genetics ; alpha 1-Antitrypsin Deficiency/therapy ; RNA, Small Interfering
    Chemical Substances Protein Aggregates ; RNA, Small Interfering
    Language English
    Publishing date 2023-07-24
    Publishing country England
    Document type Journal Article ; Review
    ZDB-ID 1182884-5
    ISSN 1744-7658 ; 0967-8298 ; 1354-3784
    ISSN (online) 1744-7658
    ISSN 0967-8298 ; 1354-3784
    DOI 10.1080/13543784.2023.2239707
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article: A gene silencing-based approach to tackle fatty liver disease.

    Strnad, Pavel / Schrader, Christina / Brunetti-Pierri, Nicola

    Molecular therapy. Methods & clinical development

    2024  Volume 32, Issue 1, Page(s) 101198

    Language English
    Publishing date 2024-02-09
    Publishing country United States
    Document type News
    ZDB-ID 2872938-9
    ISSN 2329-0501 ; 2329-0501
    ISSN (online) 2329-0501
    ISSN 2329-0501
    DOI 10.1016/j.omtm.2024.101198
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  4. Article ; Online: Pathophysiology of Chronic Liver Disease Development.

    Fromme, Malin / Strnad, Pavel

    International journal of molecular sciences

    2022  Volume 23, Issue 6

    Abstract: Chronic liver disease is a major public threat and the second leading cause of loss of working life years in Europe [ ... ]. ...

    Abstract Chronic liver disease is a major public threat and the second leading cause of loss of working life years in Europe [...].
    MeSH term(s) Europe ; Humans ; Liver Diseases/etiology
    Language English
    Publishing date 2022-03-21
    Publishing country Switzerland
    Document type Editorial
    ZDB-ID 2019364-6
    ISSN 1422-0067 ; 1422-0067 ; 1661-6596
    ISSN (online) 1422-0067
    ISSN 1422-0067 ; 1661-6596
    DOI 10.3390/ijms23063385
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  5. Article ; Online: Liver cancer in severe alpha-1 antitrypsin deficiency: who is at risk?

    Fromme, Malin / Strnad, Pavel

    The European respiratory journal

    2022  Volume 60, Issue 5

    MeSH term(s) Humans ; alpha 1-Antitrypsin Deficiency/complications ; alpha 1-Antitrypsin Deficiency/epidemiology ; Liver Neoplasms/epidemiology ; Liver Neoplasms/etiology
    Language English
    Publishing date 2022-11-03
    Publishing country England
    Document type Letter ; Comment
    ZDB-ID 639359-7
    ISSN 1399-3003 ; 0903-1936
    ISSN (online) 1399-3003
    ISSN 0903-1936
    DOI 10.1183/13993003.00718-2022
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  6. Article ; Online: Cleaning up alpha-1 antitrypsin deficiency related liver disease.

    Rademacher, Laura / Fromme, Malin / Strnad, Pavel

    Current opinion in gastroenterology

    2023  Volume 39, Issue 3, Page(s) 163–168

    Abstract: Purpose of review: Alpha-1 antitrypsin deficiency (AATD) is one of the most common genetic disorders arising due to mutations in alpha-1 antitrypsin (AAT) gene affecting primarily the lung and the liver. This review summarizes the pathophysiology and ... ...

    Abstract Purpose of review: Alpha-1 antitrypsin deficiency (AATD) is one of the most common genetic disorders arising due to mutations in alpha-1 antitrypsin (AAT) gene affecting primarily the lung and the liver. This review summarizes the pathophysiology and clinical manifestation of different AATD genotypes and discusses the recent therapeutic developments. The focus is on the severe, rare homozygous Pi∗ZZ and the common heterozygous Pi∗MZ genotype.
    Recent findings: Pi∗ZZ individuals harbor an up to 20 times higher risk of liver fibrosis and cirrhosis than noncarriers and liver transplantation is currently the only available therapeutic option. AATD constitutes a proteotoxic disorder arising from hepatic AAT accumulation and the currently most promising data come from a phase 2, open-label trial of fazirsiran, a hepatocyte-targeted siRNA. Pi∗MZ subjects display an increased risk of advanced liver disease and at the latter stage, a faster deterioration than individuals without AAT mutation.
    Summary: Although the fazirsiran data offer a glimpse of hope to AATD patients, a consensus on appropriate study endpoint, a careful patient selection as well as monitoring of long-term safety will be essential for an approval.
    MeSH term(s) Humans ; alpha 1-Antitrypsin Deficiency/complications ; alpha 1-Antitrypsin Deficiency/genetics ; Liver Cirrhosis/etiology ; Liver Cirrhosis/therapy ; Genotype ; Liver Transplantation/adverse effects
    Language English
    Publishing date 2023-03-01
    Publishing country United States
    Document type Review ; Journal Article
    ZDB-ID 632571-3
    ISSN 1531-7056 ; 0267-1379
    ISSN (online) 1531-7056
    ISSN 0267-1379
    DOI 10.1097/MOG.0000000000000919
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  7. Article ; Conference proceedings: Loss of the mechanistic target of rapamycin complexes 1 (mTORC1) causes a lethal alpha-1 antitrypsin deficiency associated liver disease

    Haber, Sophie / Lossie, Lisa / Strnad, Pavel

    Zeitschrift für Gastroenterologie

    2024  Volume 62, Issue 01

    Event/congress 40. Jahrestagung der Deutschen Arbeitsgemeinschaft zum Studium der Leber, Haus der Technik e.V., Essen, 2024-01-26
    Language German
    Publishing date 2024-01-01
    Publisher Georg Thieme Verlag
    Publishing place Stuttgart ; New York
    Document type Article ; Conference proceedings
    ZDB-ID 201387-3
    ISSN 1439-7803 ; 0044-2771 ; 0172-8504
    ISSN (online) 1439-7803
    ISSN 0044-2771 ; 0172-8504
    DOI 10.1055/s-0043-1777566
    Database Thieme publisher's database

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  8. Article ; Online: Editorial: towards an understanding of increased mortality in coeliac disease-authors' reply.

    Schneider, Carolin V / Strnad, Pavel

    Alimentary pharmacology & therapeutics

    2021  Volume 53, Issue 5, Page(s) 656

    MeSH term(s) Adult ; Celiac Disease ; Humans
    Language English
    Publishing date 2021-02-10
    Publishing country England
    Document type Editorial ; Comment
    ZDB-ID 639012-2
    ISSN 1365-2036 ; 0269-2813 ; 0953-0673
    ISSN (online) 1365-2036
    ISSN 0269-2813 ; 0953-0673
    DOI 10.1111/apt.16246
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  9. Article ; Online: SARS-CoV-2 infection in alpha1-antitrypsin deficiency.

    Schneider, Carolin V / Strnad, Pavel

    Respiratory medicine

    2021  Volume 184, Page(s) 106466

    Abstract: Alpha1-antitrypsin deficiency arises due to mutations in alpha1-antitrypsin (AAT) gene and represents the most prominent genetic predisposition to chronic obstructive pulmonary disease and emphysema. Since AAT plays important immunomodulatory and tissue- ... ...

    Abstract Alpha1-antitrypsin deficiency arises due to mutations in alpha1-antitrypsin (AAT) gene and represents the most prominent genetic predisposition to chronic obstructive pulmonary disease and emphysema. Since AAT plays important immunomodulatory and tissue-protective roles and since it was suggested to protect from severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, we assessed this association in United Kingdom Biobank, a community-based cohort with >500,000 participants. The most common, mild AATD genotypes were associated neither with increased SARS-CoV-2 infection rates nor with increased SARS-CoV-2 fatalities, while the numbers of severe AATD cases were too low to allow definitive conclusions.
    MeSH term(s) Acute Disease ; COVID-19/epidemiology ; COVID-19/mortality ; COVID-19/prevention & control ; Cohort Studies ; Female ; Genetic Predisposition to Disease/genetics ; Humans ; Male ; Mutation ; Negative Results ; Peptide Fragments/genetics ; Peptide Fragments/physiology ; Pulmonary Disease, Chronic Obstructive/genetics ; Pulmonary Emphysema/genetics ; Severity of Illness Index ; United Kingdom/epidemiology ; alpha 1-Antitrypsin/genetics ; alpha 1-Antitrypsin/physiology ; alpha 1-Antitrypsin Deficiency/genetics
    Chemical Substances C105Y peptide ; Peptide Fragments ; alpha 1-Antitrypsin
    Language English
    Publishing date 2021-05-13
    Publishing country England
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 1003348-8
    ISSN 1532-3064 ; 0954-6111
    ISSN (online) 1532-3064
    ISSN 0954-6111
    DOI 10.1016/j.rmed.2021.106466
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  10. Article ; Online: Laparoscopic Living donor liver transplantation in irresectable intrahepatic cholangiocarcinoma in primary sclerosing cholangitis associated liver cirrhosis.

    Bednarsch, Jan / Lang, Sven A / Heise, Daniel / Strnad, Pavel / Neumann, Ulf P / Ulmer, Tom F

    Zeitschrift fur Gastroenterologie

    2024  Volume 62, Issue 1, Page(s) 50–55

    Abstract: Intrahepatic cholangiocarcinoma (iCCA) is the second most common primary liver tumor and usually associated with a poor oncological prognosis. The current gold standard is the surgical resection of the tumor with subsequent adjuvant therapy. However, in ... ...

    Title translation Leberlebendspende beim technisch irresektablen intrahapatischen Cholangiokarzinom in Leberzirrhose bei primär sklerosierender Cholangitis.
    Abstract Intrahepatic cholangiocarcinoma (iCCA) is the second most common primary liver tumor and usually associated with a poor oncological prognosis. The current gold standard is the surgical resection of the tumor with subsequent adjuvant therapy. However, in case of irresectability e.g. in case of liver cirrhosis, a palliative treatment regime is conducted.This report demonstrates the case of an irresectable iCCA in liver cirrhosis due to primary sclerosing cholangitis (PSC) treated by living-donor liver transplantation (LDLT) facilitated by minimal invasive donor hepatectomy. No postoperative complications were observed in the donor and the donor was released on the 6
    MeSH term(s) Humans ; Bile Duct Neoplasms/complications ; Bile Duct Neoplasms/diagnosis ; Bile Duct Neoplasms/surgery ; Bile Ducts, Intrahepatic ; Cholangiocarcinoma/complications ; Cholangiocarcinoma/diagnosis ; Cholangiocarcinoma/surgery ; Cholangitis, Sclerosing/complications ; Cholangitis, Sclerosing/diagnosis ; Cholangitis, Sclerosing/surgery ; Laparoscopy ; Liver Cirrhosis/complications ; Liver Cirrhosis/diagnosis ; Liver Cirrhosis/surgery ; Liver Transplantation ; Living Donors ; Neoplasm Recurrence, Local
    Language English
    Publishing date 2024-01-09
    Publishing country Germany
    Document type Case Reports ; Journal Article
    ZDB-ID 201387-3
    ISSN 1439-7803 ; 0172-8504 ; 0044-2771
    ISSN (online) 1439-7803
    ISSN 0172-8504 ; 0044-2771
    DOI 10.1055/a-2221-6126
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