Michael Kutsche

Universität Hamburg

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Werner Klipp

Ruhr University Bochum

Melitta Schachner

Rutgers, The State University of New Jersey

Theresia Herget

Universität Hamburg

Kerstin Kutsche

Universität Hamburg

Kevin Paul

Universität Hamburg

Melitta Schachner

Rutgers, The State University of New Jersey

Christian Kubisch

University Medical Center Hamburg-Eppendorf

H. Gregg Schuler

Universität Hamburg

Sieglinde Angermüller

Friedrich-Alexander-Universität Erlangen-Nürnberg

Dzhoy Papingi

Universität Hamburg

Cooperative Institutions

Universität Hamburg

University Medical Center Hamburg-Eppendorf

Heidelberg University

Children's Hospital Central California

Children's Hospital of Philadelphia

Charité - Universitätsmedizin Berlin

University Hospital Heidelberg

Children's Hospital of Los Angeles

Harvard University

Bielefeld University

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Pilot Study: Maternal Betamethasone Administration Has Long Term Effects on Umbilical Cord Glucose Levels in Human Fetuses

Michael Kutsche A Husar L. Ehrlich D. M. Sloboda John Challis

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Thoracic Aortic Disease in Patients with Heterozygous Variants in FBN2

The Thoracic and Cardiovascular Surgeon (2022)

Till Demal Marco Sachse H. Gregg Schuler H. Reichenspurner Michael Kutsche

Background: Inherited thoracic aortic disease defines a group of disorders characterized by aortic aneurysm or dissection and high mortality. The most common genetic causes of thoracic aortic disease are heterozygous pathogenic variants in the FBN1 and ACTA2 genes. Heterozygous FBN2 variants have rarely been associated with aortic disease. Pathogenic variants in the central region (exons 24–35) of FBN2 cause a hereditary connective tissue disorder named congenital contractual arachnodactyly (CCA).

Arachnodactyly

Thoracic aortic aneurysm

Genetic disorder

Heterozygote advantage

10.1055/s-0042-1742951

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Functional analysis of the cysteine motifs in the ferredoxin-like protein FdxN ofRhizobium meliloti involved in symbiotic nitrogen fixation

MGG Molecular & General Genetics (1992)

Bernd Masepohl Michael Kutsche Kai-Uwe Riedel Manfred Schmehl Werner Klipp

10.1007/bf00587558

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Citations (23)

Regulation of Molybdenum and Alternative Nitrogenases in the Photosynthetic Purple Bacterium Rhodobacter Capsulatus

Current plant science and biotechnology in agriculture (1995)

Werner Klipp Sieglinde Angermüller S. Astroth Paul-Bertram Kamp Manfred Kern

Rhodobacter

Purple bacteria

Sigma factor

10.1007/978-94-011-0379-4_28

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Citations (3)

Elsevier eBooks (2009)

Alex Broeckel Carlos Cabrera Chee Ming Wong Chris Thunig Daarken

10.1016/b978-0-240-52174-9.50057-9

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Cancer incidence and spectrum among children with genetically confirmed Beckwith-Wiedemann spectrum in Germany: a retrospective cohort study

British Journal of Cancer (2020)

Sümeyye Cöktü Claudia Spix Melanie Kaiser Jasmin Beygo Stephanie Kleinle

Beckwith-Wiedemann syndrome (BWS) is a cancer predisposition syndrome caused by defects on chromosome 11p15.5. The quantitative cancer risks in BWS patients depend on the underlying (epi)genotype but have not yet been assessed in a population-based manner.We identified a group of 321 individuals with a molecularly confirmed diagnosis of BWS and analysed the cancer incidence up to age 15 years and cancer spectrum by matching their data with the German Childhood Cancer Registry.We observed 13 cases of cancer in the entire BWS cohort vs 0.4 expected. This corresponds to a 33-fold increased risk (standardised incidence ratio (SIR) = 32.6; 95% confidence interval = 17.3-55.7). The specific cancers included hepatoblastoma (n = 6); nephroblastoma (n = 4); astrocytoma (n = 1); neuroblastoma (n = 1) and adrenocortical carcinoma (n = 1). The cancer SIR was highest in patients with a paternal uniparental disomy of 11p15.5 (UPDpat). A high cancer risk remained when cases of cancer diagnosed prior to the BWS diagnosis were excluded.This study confirms an increased cancer risk in children with BWS. Our findings suggest that the highest cancer risk is associated with UPDpat. We were unable to confirm an excessive cancer risk in patients with IC1 gain of methylation (IC1-GOM) and this finding requires further investigation.

Standardized mortality ratio

Hepatoblastoma

10.1038/s41416-020-0911-x

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Citations (41)

Promoters controlling expression of the alternative nitrogenase and the molybdenum uptake system in Rhodobacter capsulatus are activated by NtrC, independent of sigma54, and repressed by molybdenum

Journal of Bacteriology (1996)

Michael Kutsche Silke Leimkühler Sieglinde Angermüller Werner Klipp

The alternative nitrogenase of Rhodobacter capsulatus is expressed only under conditions of nitrogen and molybdenum depletion. The analysis of anfA-lacZ fusions demonstrated that this dual control occurred at the level of transcription of anfA, which encodes a transcriptional activator specific for the alternative nitrogenase. The anfA promoter was found to be activated under nitrogen-limiting conditions by NtrC in a sigma54-independent manner. In addition, anfA transcription was repressed by traces of molybdenum. This molybdenum-dependent repression of anfA was released in R. capsulatus mutants carrying either lesions in the high-affinity molybdenum uptake system (modABCD) or a double deletion of mopA and mopB, two genes encoding molybdenum-pterin-binding proteins. The expression of the molybdenum transport system itself was shown to be negatively regulated by molybdenum and, unexpectedly, to be also regulated by NtrC. This finding is in line with the presence of two tandemly arranged DNA motifs located in front of the R. capsulatus mopA-modABCD operon, which are homologous to R. capsulatus NtrC binding sites. Mapping of the transcriptional initiation sites of mopA and anfA revealed promoter sequences exhibiting significant homology to each other but no homology to known prokaryotic promoters. In addition, a conserved DNA sequence of dyad symmetry overlapping the transcriptional initiation sites of mopA and anfA was found. Deletions within this element resulted in molybdenum-independent expression of anfA, indicating that this DNA sequence may be the target of MopA/MopB-mediated repression.

Rhodobacter

Transcription

10.1128/jb.178.7.2010-2017.1996

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Citations (71)

Reduced GABAergic transmission and number of hippocampal perisomatic inhibitory synapses in juvenile mice deficient in the neural cell adhesion molecule L1

Molecular and Cellular Neuroscience (2004)

Armen Saghatelyan Alexander G. Nikonenko Mu Sun Bettina Rolf Peggy Putthoff

Pyramidal cell

Postsynaptic Current

10.1016/j.mcn.2004.01.008

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Citations (71)

A novel TTC26 variant in a patient with hexadactyly, pituitary stalk interruption, hepatopathy, nephropathy, and bilateral lip‐palate cleft: A case report and expansion of the phenotype

American Journal of Medical Genetics Part A (2023)

Dzhoy Papingi Tatjana Bierhals Alexander E. Volk Michael Kutsche Kevin Paul

Abstract Biallelic pathogenic variants in the TTC26 gene are known to cause BRENS (biliary, renal, neurological, skeletal) syndrome, an ultra‐rare autosomal recessive condition with only few patients published to date. BRENS syndrome is characterized by hexadactyly, severe neonatal cholestasis, and involvement of the brain, heart, and kidney, however the full phenotypic and genotypic spectrum is unknown. Here, we report on a previously undescribed homozygous intronic TTC26 variant (c.1006‐5 T > C) in a patient showing some of the known TTC26 ‐associated features like hexadactyly, hypopituitarism, hepatopathy, nephropathy, and congenital heart defect. Moreover, he presented with a suspected unilateral hearing loss and bilateral cleft lip‐palate. The variant is considered to affect correct splicing by the loss of the canonical acceptor splice site and activation of a cryptic acceptor splice site. Hereby, our patient represents one additional patient with BRENS syndrome carrying a previously unreported TTC26 variant. Furthermore, we confirm the involvement of the pituitary gland to be a common clinical feature of the syndrome and broaden the clinical spectrum of TTC26 ciliopathy to include facial clefts and a probable hearing involvement.

Ciliopathy

10.1002/ajmg.a.63515

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Citations (1)

Neural cell adhesion molecule L1 is required for fasciculation and routing of thalamocortical fibres and corticothalamic fibres

Neuroscience Research (2004)

Kyoji Ohyama Kyoko Tan‐Takeuchi Michael Kutsche Melitta Schachner Keiichi Uyemura

Fasciculation

10.1016/j.neures.2003.12.011

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Citations (30)