Modelling Decentralized Hydrogen Systems: Lessons Learned and Challenges from German Regions

Green hydrogen produced by power-to-gas will play a major role in the defossilization of the energy system as it offers both carbon-neutral chemical energy and the chance to provide flexibility. This paper provides an extensive analysis of hydrogen production in decentralized energy systems, as well as possible operation modes (H2 generation or system flexibility). Modelling was realized for municipalities—the lowest administrative unit in Germany, thus providing high spatial resolution—in the linear optimization framework OEMOF. The results allowed for a detailed regional analysis of the specific operating modes and were analyzed using full-load hours, share of used negative residual load, installed capacity and levelized cost of hydrogen to derive the operation mode of power-to-gas to produce hydrogen. The results show that power-to-gas is mainly characterized by constant hydrogen production and rarely provides flexibility to the system. Main drivers of this dominant operation mode include future demand for hydrogen and the fact that high full-load hours reduce hydrogen-production costs. However, changes in the regulatory, market and technical framework could promote more flexibility and support possible use cases for the central technology to succeed in the energy transition.BMBF, 03SFK4N0, Verbundvorhaben ENavi: Energiewende-Navigationssystem zur Erfassung, Analyse und Simulation der systemischen Vernetzungen" - Teilvorhaben N0BMBF, 03SFK4Y0, Verbundvorhaben ENavi: Energiewende-Navigationssystem zur Erfassung, Analyse und Simulation der systemischen Vernetzungen - Teilvorhaben Y

Mutations in SPAG1 Cause Primary Ciliary Dyskinesia Associated with Defective Outer and Inner Dynein Arms

Primary ciliary dyskinesia (PCD) is a genetically heterogeneous, autosomal-recessive disorder, characterized by oto-sino-pulmonary disease and situs abnormalities. PCD-causing mutations have been identified in 20 genes, but collectively they account for only ∼65% of all PCDs. To identify mutations in additional genes that cause PCD, we performed exome sequencing on three unrelated probands with ciliary outer and inner dynein arm (ODA+IDA) defects. Mutations in SPAG1 were identified in one family with three affected siblings. Further screening of SPAG1 in 98 unrelated affected individuals (62 with ODA+IDA defects, 35 with ODA defects, 1 without available ciliary ultrastructure) revealed biallelic loss-of-function mutations in 11 additional individuals (including one sib-pair). All 14 affected individuals with SPAG1 mutations had a characteristic PCD phenotype, including 8 with situs abnormalities. Additionally, all individuals with mutations who had defined ciliary ultrastructure had ODA+IDA defects. SPAG1 was present in human airway epithelial cell lysates but was not present in isolated axonemes, and immunofluorescence staining showed an absence of ODA and IDA proteins in cilia from an affected individual, thus indicating that SPAG1 probably plays a role in the cytoplasmic assembly and/or trafficking of the axonemal dynein arms. Zebrafish morpholino studies of spag1 produced cilia-related phenotypes previously reported for PCD-causing mutations in genes encoding cytoplasmic proteins. Together, these results demonstrate that mutations in SPAG1 cause PCD with ciliary ODA+IDA defects and that exome sequencing is useful to identify genetic causes of heterogeneous recessive disorders

Production of He-4 and (4) in Pb-Pb collisions at root(NN)-N-S=2.76 TeV at the LHC

Results on the production of He-4 and (4) nuclei in Pb-Pb collisions at root(NN)-N-S = 2.76 TeV in the rapidity range vertical bar y vertical bar <1, using the ALICE detector, are presented in this paper. The rapidity densities corresponding to 0-10% central events are found to be dN/dy4(He) = (0.8 +/- 0.4 (stat) +/- 0.3 (syst)) x 10(-6) and dN/dy4 = (1.1 +/- 0.4 (stat) +/- 0.2 (syst)) x 10(-6), respectively. This is in agreement with the statistical thermal model expectation assuming the same chemical freeze-out temperature (T-chem = 156 MeV) as for light hadrons. The measured ratio of (4)/He-4 is 1.4 +/- 0.8 (stat) +/- 0.5 (syst). (C) 2018 Published by Elsevier B.V.Peer reviewe

Mutations in SPAG1 Cause Primary Ciliary Dyskinesia Associated with Defective Outer and Inner Dynein Arms

Primary ciliary dyskinesia (PCD) is a genetically heterogeneous, autosomal-recessive disorder, characterized by oto-sino-pulmonary disease and situs abnormalities. PCD-causing mutations have been identified in 20 genes, but collectively they account for only ∼65% of all PCDs. To identify mutations in additional genes that cause PCD, we performed exome sequencing on three unrelated probands with ciliary outer and inner dynein arm (ODA+IDA) defects. Mutations in SPAG1 were identified in one family with three affected siblings. Further screening of SPAG1 in 98 unrelated affected individuals (62 with ODA+IDA defects, 35 with ODA defects, 1 without available ciliary ultrastructure) revealed biallelic loss-of-function mutations in 11 additional individuals (including one sib-pair). All 14 affected individuals with SPAG1 mutations had a characteristic PCD phenotype, including 8 with situs abnormalities. Additionally, all individuals with mutations who had defined ciliary ultrastructure had ODA+IDA defects. SPAG1 was present in human airway epithelial cell lysates but was not present in isolated axonemes, and immunofluorescence staining showed an absence of ODA and IDA proteins in cilia from an affected individual, thus indicating that SPAG1 probably plays a role in the cytoplasmic assembly and/or trafficking of the axonemal dynein arms. Zebrafish morpholino studies of spag1 produced cilia-related phenotypes previously reported for PCD-causing mutations in genes encoding cytoplasmic proteins. Together, these results demonstrate that mutations in SPAG1 cause PCD with ciliary ODA+IDA defects and that exome sequencing is useful to identify genetic causes of heterogeneous recessive disorders

DYX1C1 is required for axonemal dynein assembly and ciliary motility

DYX1C1 has been associated with dyslexia and neuronal migration in the developing neocortex. Unexpectedly, we found that deleting exons 2–4 of Dyx1c1 in mice caused a phenotype resembling primary ciliary dyskinesia (PCD), a disorder characterized by chronic airway disease, laterality defects and male infertility. This phenotype was confirmed independently in mice with a Dyx1c1 c.T2A start-codon mutation recovered from an N-ethyl-N-nitrosourea (ENU) mutagenesis screen. Morpholinos targeting dyx1c1 in zebrafish also caused laterality and ciliary motility defects. In humans, we identified recessive loss-of-function DYX1C1 mutations in 12 individuals with PCD. Ultrastructural and immunofluorescence analyses of DYX1C1-mutant motile cilia in mice and humans showed disruptions of outer and inner dynein arms (ODAs and IDAs, respectively). DYX1C1 localizes to the cytoplasm of respiratory epithelial cells, its interactome is enriched for molecular chaperones, and it interacts with the cytoplasmic ODA and IDA assembly factor DNAAF2 (KTU). Thus, we propose that DYX1C1 is a newly identified dynein axonemal assembly factor (DNAAF4)

Modelling Decentralized Hydrogen Systems: Lessons Learned and Challenges from German Regions

Green hydrogen produced by power-to-gas will play a major role in the defossilization of the energy system as it offers both carbon-neutral chemical energy and the chance to provide flexibility. This paper provides an extensive analysis of hydrogen production in decentralized energy systems, as well as possible operation modes (H2 generation or system flexibility). Modelling was realized for municipalities&mdash;the lowest administrative unit in Germany, thus providing high spatial resolution&mdash;in the linear optimization framework OEMOF. The results allowed for a detailed regional analysis of the specific operating modes and were analyzed using full-load hours, share of used negative residual load, installed capacity and levelized cost of hydrogen to derive the operation mode of power-to-gas to produce hydrogen. The results show that power-to-gas is mainly characterized by constant hydrogen production and rarely provides flexibility to the system. Main drivers of this dominant operation mode include future demand for hydrogen and the fact that high full-load hours reduce hydrogen-production costs. However, changes in the regulatory, market and technical framework could promote more flexibility and support possible use cases for the central technology to succeed in the energy transition

Data for "A power-based abrasion law for use in landscape evolution models"

Experimental data used in the paper: "A power-based abrasion law for use in landscape evolution models," published in Geology.Subglacial abrasion drives erosion for many glaciers, inundating forefields and proglacial marine environments with glaciogenic sediments. Theoretical treatments of this process suggest that bedrock abrasion rates scale linearly with the energy expended through rock-on-rock friction during slip, but this assumption lacks an empirical basis for general implementation. To test this approach, we simulated abrasion by sliding debris-laden ice over rock beds under subglacial conditions in a cryo-ring shear and a direct shear device. Miniscule volumes of erosion that occurred during each run were mapped with a white-light profilometer, and we measured the rock mechanical properties needed to constrain the abrasion energy expenditure. We find that abraded volume per unit area increases linearly with average shear force at the bed and that abrasion rates increase linearly with basal power for plane beds. Lastly, only a small percentage (⪅1%) of the energy partitioned to basal slip is dissipated by abrasion. These results confirm the basal-power abrasion rule is viable to implement in landscape evolution models.This work is supported by NSF 2017185

Characteristics and Prognostic Relevance of Ventricular Arrhythmia in Patients with Myocarditis

Myocarditis is characterized by various clinical manifestations, with ventricular arrhythmia (VA) as a frequent symptom at initial presentation. Here, we investigated characteristics and prognostic relevance of VA in patients with myocarditis. The study population consisted of 76 patients with myocarditis, verified by biopsy and/or cardiac magnetic resonance (CMR) imaging, including 38 consecutive patients with VA (45 &plusmn; 3 years, 68% male) vs. 38 patients without VA (NVA) (38 &plusmn; 2 years, 84% male) serving as a control group. VA was monomorphic ventricular tachycardia in 55% of patients, premature ventricular complexes in 50% and ventricular fibrillation in 29%. The left ventricular ejection fraction at baseline was 47 &plusmn; 2% vs. 40 &plusmn; 3% in VA vs. NVA patients (p = 0.069). CMR showed late gadolinium enhancement more often in VA patients (94% vs. 69%; p = 0.016), incorporating 17.6 &plusmn; 1.8% vs. 8.2 &plusmn; 1.3% of myocardial mass (p &lt; 0.001). Radiofrequency catheter ablation for VA was initially performed in nine (24%) patients, of whom five remained free from any recurrence over 24 &plusmn; 3 months. Taken together, in patients with myocarditis, reduced left ventricular ejection fraction does not predict VA occurrence but CMR shows late gadolinium enhancement more frequently and to a larger extent in VA than in NVA patients, potentially guiding catheter ablation as a reasonable treatment of VA in this population

Acute impact of an endurance race on biventricular and biatrial myocardial strain in competitive male and female triathletes evaluated by feature-tracking CMR

Objectives!#!Cardiac adaptation in endurance athletes is a well-known phenomenon, but the acute impact of strenuous exercise is rarely reported on. The aim of this study was to analyze the alterations in biventricular and biatrial function in triathletes after an endurance race using novel feature-tracking cardiac magnetic resonance (FT-CMR).!##!Methods!#!Fifty consecutive triathletes (45 ± 10 years; 80% men) and twenty-eight controls were prospectively recruited, and underwent 1.5-T CMR. Biventricular and biatrial volumes, left ventricular ejection fraction (LVEF), FT-CMR analysis, and late gadolinium imaging (LGE) were performed. Global systolic longitudinal (GLS), circumferential (GCS), and radial strain (GRS) were assessed. CMR was performed at baseline and following an endurance race. High-sensitive troponin T and NT-proBNP were determined. The time interval between race completion and CMR was 2.3 ± 1.1 h (range 1-5 h).!##!Results!#!Post-race troponin T (p &amp;lt; 0.0001) and NT-proBNP (p &amp;lt; 0.0001) were elevated. LVEF remained constant (62 ± 6 vs. 63 ± 7%, p = 0.607). Post-race LV GLS decreased by tendency (- 18 ± 2 vs. - 17 ± 2%, p = 0.054), whereas GCS (- 16 ± 4 vs. - 18 ± 4%, p &amp;lt; 0.05) and GRS increased (39 ± 11 vs. 44 ± 11%, p &amp;lt; 0.01). Post-race right ventricular GLS (- 19 ± 3 vs. - 19 ± 3%, p = 0.668) remained constant and GCS increased (- 7 ± 2 vs. - 8 ± 3%, p &amp;lt; 0.001). Post-race left atrial GLS (30 ± 8 vs. 24 ± 6%, p &amp;lt; 0.0001) decreased while right atrial GLS remained constant (25 ± 6 vs. 24 ± 6%, p = 0.519).!##!Conclusions!#!The different alterations of post-race biventricular and biatrial strain might constitute an intrinsic compensatory mechanism following an acute bout of endurance exercise. The combined use of strain parameters may allow a better characterization of ventricular and atrial function in endurance athletes.!##!Key points!#!• Triathletes demonstrate a decrease of LV global longitudinal strain by tendency and constant RV global longitudinal strain following an endurance race. • Post-race LV and RV global circumferential and radial strains increase, possibly indicating a compensatory mechanism after an acute endurance exercise bout. • Subgroup analyses of male triathletes with focal myocardial fibrosis did not demonstrate alterations in biventricular and biatrial strain after an endurance race