Sabine Andrea Smolorz

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About Sabine Andrea Smolorz:

No description specified

SEEK ID: https://goeseek.gwdguser.de/people/14

Location: Germany

ORCID: https://orcid.org/0000-0002-6184-3891

Joined: 17th Apr 2023

Expertise: Data Management, Molecular Biology, Genetics

Tools: Not specified

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Default programme - for test purposes

For test purposes only.

Projects: Default Project - for test purposes, TAVI test project

Web page: Not specified

Cluster of Excellence

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Projects: MBExC: Multiscale Bioimaging

Web page: https://www.wikidata.org/wiki/Q48975698

Other research project backgrounds

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Projects: Cardiomyocyte tissues, 12 months EHM project (Dittrich, Zimmermann)

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Sonderforschungsbereiche/Collaborative Research Centers

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Projects: CRC1002: Modulatory Units in Heart Failure, TRR 274: Checkpoints of Central Nervous System Recovery, SFB 1190: Compartmental Gates and Contact Sites in Cells

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Showing 8 out of a possible 9 Projects Advanced Projects list for this Person with search and filtering

12 months EHM project (Dittrich, Zimmermann)

Engineered human myocardium (EHM) is cultivated for up to 12 months and analyzed via different (OMICs) techniques to create a reference dataset for further . Primary researcher is Gesine M. Dittrich from the department of Pharmacology and Toxicology (UMG, head: Prof. W.-H. Zimmermann).

Programme: Other research project backgrounds

Public web page: Not specified

Organisms: Human

Default Project - for test purposes

No description specified

Programme: Default programme - for test purposes

Public web page: Not specified

Organisms: Not specified

CRC 1565: Molecular mechanisms and interplay of gene expression processes

https://gepris.dfg.de/gepris/projekt/469281184

While gene expression was traditionally viewed as a linear pathway of consecutive steps, we now know that genome organization and transcription, RNA processing and modification, RNA-protein complex assembly, translation and quality control are highly interconnected processes. Many cross-regulation events occur even between non-consecutive steps of gene expression, and even between processes taking place in different cellular compartments.

This paradigm ...

Programme: This Project is not associated with a Programme

Public web page: https://sfb1565.uni-goettingen.de/

Organisms: Not specified

MBExC: Multiscale Bioimaging

https://mbexc.uni-goettingen.de/

Programme: Cluster of Excellence

Public web page: https://mbexc.de/

Organisms: Human, Mouse, Rat, Saccharomyces cerevisiae (Yeast)

TAVI test project

This is a project to test the functionalities of SEEK for the storage of research outputs generated from biobank samples collected from TAVI patients.

Programme: Default programme - for test purposes

Public web page: Not specified

Organisms: Human

SFB 1190: Compartmental Gates and Contact Sites in Cells

https://gepris.dfg.de/gepris/projekt/264061860

https://sfb1190.de/

https://sfb1190.med.uni-goettingen.de/

Programme: Sonderforschungsbereiche/Collaborative Research Centers

Public web page: https://sfb1190.de/

Organisms: Human, Mouse, Saccharomyces cerevisiae (Yeast)

CRC1002: Modulatory Units in Heart Failure

https://sfb1002.med.uni-goettingen.de/ (Research Data Platform) https://gepris.dfg.de/gepris/projekt/193793266?language=en (DFG Gepris entry) With a prevalence of between two and four percent, heart failure remains one of the most common diseases in Europe. Although tremendous progress has been made in its treatment, the morbidity and mortality of affected patients remains high. Advanced heart failure can still only be treated palliatively.We define modulatory units as cell-based and/or genetic ...

Programme: Sonderforschungsbereiche/Collaborative Research Centers

Public web page: https://herzzentrum.umg.eu/forschung/forschungsverbuende/sfb-1002/

Start date: 1st Jul 2012

End date: 30th Jun 2024

Organisms: Human, Mouse

Cardiomyocyte tissues

No description specified

Programme: Other research project backgrounds

Public web page: Not specified

Organisms: ACTN2-Citrine Cardiomyocytes, Human, Mouse

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University Medical Center Göttingen

Country: Germany

City: Göttingen

Web page: https://ror.org/021ft0n22

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Engineered skeletal muscle recapitulates human muscle development, regeneration and dystrophy.

CRC1002: Modulatory Units in Heart Failure

Abstract (Expand)

BACKGROUND: Human pluripotent stem cell-derived muscle models show great potential for translational research. Here, we describe developmentally inspired methods for the derivation of skeletal muscle … cells and their utility in skeletal muscle tissue engineering with the aim to model skeletal muscle regeneration and dystrophy in vitro. METHODS: Key steps include the directed differentiation of human pluripotent stem cells to embryonic muscle progenitors followed by primary and secondary foetal myogenesis into three-dimensional muscle. To simulate Duchenne muscular dystrophy (DMD), a patient-specific induced pluripotent stem cell line was compared to a CRISPR/Cas9-edited isogenic control line. RESULTS: The established skeletal muscle differentiation protocol robustly and faithfully recapitulates critical steps of embryonic myogenesis in two-dimensional and three-dimensional cultures, resulting in functional human skeletal muscle organoids (SMOs) and engineered skeletal muscles (ESMs) with a regeneration-competent satellite-like cell pool. Tissue-engineered muscle exhibits organotypic maturation and function (up to 5.7 +/- 0.5 mN tetanic twitch tension at 100 Hz in ESM). Contractile performance could be further enhanced by timed thyroid hormone treatment, increasing the speed of contraction (time to peak contraction) as well as relaxation (time to 50% relaxation) of single twitches from 107 +/- 2 to 75 +/- 4 ms (P < 0.05) and from 146 +/- 6 to 100 +/- 6 ms (P < 0.05), respectively. Satellite-like cells could be documented as largely quiescent PAX7(+) cells (75 +/- 6% Ki67(-) ) located adjacent to muscle fibres confined under a laminin-containing basal membrane. Activation of the engineered satellite-like cell niche was documented in a cardiotoxin injury model with marked recovery of contractility to 57 +/- 8% of the pre-injury force 21 days post-injury (P < 0.05 compared to Day 2 post-injury), which was completely blocked by preceding irradiation. Absence of dystrophin in DMD ESM caused a marked reduction of contractile force (-35 +/- 7%, P < 0.05) and impaired expression of fast myosin isoforms resulting in prolonged contraction (175 +/- 14 ms, P < 0.05 vs. gene-edited control) and relaxation (238 +/- 22 ms, P < 0.05 vs. gene-edited control) times. Restoration of dystrophin levels by gene editing rescued the DMD phenotype in ESM. CONCLUSIONS: We introduce human muscle models with canonical properties of bona fide skeletal muscle in vivo to study muscle development, maturation, disease and repair.

Authors: M. Shahriyari, M. R. Islam, S. M. Sakib, M. Rinn, A. Rika, D. Kruger, L. Kaurani, V. Gisa, M. Winterhoff, H. Anandakumar, O. Shomroni, M. Schmidt, G. Salinas, A. Unger, W. A. Linke, J. Zschuntzsch, J. Schmidt, R. Bassel-Duby, E. N. Olson, A. Fischer, W. H. Zimmermann, M. Tiburcy

Date Published: 19th Oct 2022

Publication Type: Journal

PubMed ID: 36254806

Citation: J Cachexia Sarcopenia Muscle. 2022 Dec;13(6):3106-3121. doi: 10.1002/jcsm.13094. Epub 2022 Oct 18.

Created: 26th May 2023 at 11:43

Intronic CRISPR Repair in a Preclinical Model of Noonan Syndrome-Associated Cardiomyopathy.

CRC1002: Modulatory Units in Heart Failure

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Abstract (Expand)

BACKGROUND: Noonan syndrome (NS) is a multisystemic developmental disorder characterized by common, clinically variable symptoms, such as typical facial dysmorphisms, short stature, developmental delay, … intellectual disability as well as cardiac hypertrophy. The underlying mechanism is a gain-of-function of the RAS-mitogen-activated protein kinase signaling pathway. However, our understanding of the pathophysiological alterations and mechanisms, especially of the associated cardiomyopathy, remains limited and effective therapeutic options are lacking. METHODS: Here, we present a family with two siblings displaying an autosomal recessive form of NS with massive hypertrophic cardiomyopathy as clinically the most prevalent symptom caused by biallelic mutations within the leucine zipper-like transcription regulator 1 (LZTR1). We generated induced pluripotent stem cell-derived cardiomyocytes of the affected siblings and investigated the patient-specific cardiomyocytes on the molecular and functional level. RESULTS: Patients' induced pluripotent stem cell-derived cardiomyocytes recapitulated the hypertrophic phenotype and uncovered a so-far-not-described causal link between LZTR1 dysfunction, RAS-mitogen-activated protein kinase signaling hyperactivity, hypertrophic gene response and cellular hypertrophy. Calcium channel blockade and MEK inhibition could prevent some of the disease characteristics, providing a molecular underpinning for the clinical use of these drugs in patients with NS, but might not be a sustainable therapeutic option. In a proof-of-concept approach, we explored a clinically translatable intronic CRISPR (clustered regularly interspaced short palindromic repeats) repair and demonstrated a rescue of the hypertrophic phenotype. CONCLUSIONS: Our study revealed the human cardiac pathogenesis in patient-specific induced pluripotent stem cell-derived cardiomyocytes from NS patients carrying biallelic variants in LZTR1 and identified a unique disease-specific proteome signature. In addition, we identified the intronic CRISPR repair as a personalized and in our view clinically translatable therapeutic strategy to treat NS-associated hypertrophic cardiomyopathy.

Authors: U. Hanses, M. Kleinsorge, L. Roos, G. Yigit, Y. Li, B. Barbarics, I. El-Battrawy, H. Lan, M. Tiburcy, R. Hindmarsh, C. Lenz, G. Salinas, S. Diecke, C. Muller, I. Adham, J. Altmuller, P. Nurnberg, T. Paul, W. H. Zimmermann, G. Hasenfuss, B. Wollnik, L. Cyganek

Date Published: 15th Sep 2020

Publication Type: Journal

PubMed ID: 32623905

Citation: Circulation. 2020 Sep 15;142(11):1059-1076. doi: 10.1161/CIRCULATIONAHA.119.044794. Epub 2020 Jul 6.

Created: 30th May 2023 at 14:27

Extracellular Vesicles Derived from Neural Progenitor Cells--a Preclinical Evaluation for Stroke Treatment in Mice.

CRC1002: Modulatory Units in Heart Failure

Abstract (Expand)

Stem cells such as mesenchymal stem cells (MSCs) enhance neurological recovery in preclinical stroke models by secreting extracellular vesicles (EVs). Since previous reports have focused on the application …

Authors: X. Zheng, L. Zhang, Y. Kuang, V. Venkataramani, F. Jin, K. Hein, M. P. Zafeiriou, C. Lenz, W. Moebius, E. Kilic, D. M. Hermann, M. S. Weber, H. Urlaub, W. H. Zimmermann, M. Bahr, T. R. Doeppner

Date Published: 4th May 2020

Publication Type: Journal

PubMed ID: 32361827

Citation: Transl Stroke Res. 2021 Feb;12(1):185-203. doi: 10.1007/s12975-020-00814-z. Epub 2020 May 2.

Created: 24th May 2023 at 15:28

CRISPLD1: a novel conserved target in the transition to human heart failure.

CRC1002: Modulatory Units in Heart Failure

Abstract (Expand)

Heart failure is a major health problem worldwide with a significant morbidity and mortality rate. Although studied extensively in animal models, data from patients at the compensated disease stage are …

Authors: S. Khadjeh, V. Hindmarsh, F. Weber, L. Cyganek, R. O. Vidal, S. Torkieh, K. Streckfuss-Bomeke, D. Lbik, M. Tiburcy, B. A. Mohamed, S. Bonn, K. Toischer, G. Hasenfuss

Date Published: 7th Mar 2020

Publication Type: Journal

PubMed ID: 32146539

Citation: Basic Res Cardiol. 2020 Mar 7;115(3):27. doi: 10.1007/s00395-020-0784-4.

Created: 30th May 2023 at 12:34

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