Cobalt Polypyridyl-Based Electrolytes for p-Type Dye-Sensitized Solar Cells

Polypyridyl Co complexes with different substituents were applied as redox mediators in p-type dye-sensitized solar cells (p-DSCs), consisting of mesoporous NiO sensitized with a perylenemonoimide-naphthalenediimide (PMI-NDI) dyad. The photocurrent and photovoltages of the devices were found to depend on the steric bulk of the redox species rather than their electrochem. potential. Bulky substituents were found to slow the detrimental charge recombination reactions between holes in the NiO semiconductor and the reduced form of the redox couple. The open-circuit potential (VOC) of each of the devices was superior to the equiv. PMI-NDI-sensitized p-DSCs contg. the triiodide/iodide redox couple

Mimicking Photosystem I with a Transmembrane Light Harvester and Energy Transfer‐Induced Photoreduction in Phospholipid Bilayers

Photosystem I (PS I) is a transmembrane protein that assembles perpendicular to the membrane, and performs light harvesting, energy transfer, and electron transfer to a final, water-soluble electron acceptor. We present here a supramolecular model of it formed by a bicationic oligofluorene 1(2+) bound to the bisanionic photoredox catalyst eosin Y (EY2-) in phospholipid bilayers. According to confocal microscopy, molecular modeling, and time dependent density functional theory calculations, 1(2+) prefers to align perpendicularly to the lipid bilayer. In presence of EY2-, a strong complex is formed (K-a=2.1 +/- 0.1x10(6) m(-1)), which upon excitation of 1(2+) leads to efficient energy transfer to EY2-. Follow-up electron transfer from the excited state of EY2- to the water-soluble electron donor EDTA was shown via UV-Vis absorption spectroscopy. Overall, controlled self-assembly and photochemistry within the membrane provides an unprecedented yet simple synthetic functional mimic of PS I.Metals in Catalysis, Biomimetics & Inorganic Material

DCLRE1C (ARTEMIS) mutations causing phenotypes ranging from atypical severe combined immunodeficiency to mere antibody deficiency

Null mutations in genes involved in V(D)J recombination cause a block in B- and T- cell development, clinically presenting as severe combined immunodeficiency (SCID). Hypomorphic mutations in the non-homologous end joining gene DCLRE1C (encoding ARTEMIS) have been described to cause atypical SCID, Omenn syndrome, Hyper IgM syndrome and inflammatory bowel disease - all with severely impaired T-cell immunity. By whole-exome sequencing, we investigated the molecular defect in a consanguineous family with three children clinically diagnosed with antibody deficiency. We identified perfectly segregating homozygous variants in DCLRE1C in three index patients with recurrent respiratory tract infections, very low B cell numbers and serum IgA levels. In patients, decreased colony survival after irradiation, impaired proliferative response, and reduced counts of naïve T cells were observed in addition to a restricted T cell receptor repertoire, increased palindromic nucleotides in the complementarity determining regions 3, and long stretches of microhomology at switch junctions. Defective V(D)J recombination was complemented by wild-type ARTEMIS protein in vitro. Subsequently, homozygous or compound heterozygous DCLRE1C mutations were identified in nine patients from the same geographic region. We demonstrate that DCLRE1C mutations can cause a phenotype presenting as only antibody deficiency. This novel association broadens the clinical spectrum associated with ARTEMIS mutations. Clinicians should consider the possibility that an immunodeficiency with a clinically mild initial presentation could be a combined immunodeficiency, so as to provide appropriate care for affected patients

Study of the bunch crossing identification at LHC using microstrip gas chambers

none47During the beam test of a tracker prototype for the Compact Muon Solenoid detector proposed for the LHC, the time response of the Microstrip Gas Chambers was studied using different gases and chamber gaps. The subsequent efficiency to identify the bunch crossing at LHC is discussed for several algorithms used in the off-line signal processing of the data.F. Angelini;N. Bacchetta;R. Bellazzini;D. Bon;A. Bondar;M. Bozzo;A. Brez;J. Brom;J. Caldero;J. Clergeau;D. Contardo;M. French;A. Giraldo;G. Guillot;G. Hall;R. Hammarström;R. Haroutunian;L. Jones;T. Kachelhoffer;D. Kryn;M. Loreti;T. k´Ladziński;J. Mabo;B. MacEvoy;M. Massai;M. Miguet;M. Millmore;A. Morelli;V. Nagaslaev;A. Pallares;F. Parodi;A. Peisert;S. Quian;F. Raffo;M. Raymond;M. Rebouillat;J. Riester;O. Runolfsson;R. Sachdeva;L. Shekhtman;G. Smadja;G. Spandre;M. Spezziga;A. Toropin;C. V. Velde;P. Vanlaer;D. VitèF., Angelini; N., Bacchetta; R., Bellazzini; D., Bon; A., Bondar; Bozzo, Marco; A., Brez; J., Brom; J., Caldero; J., Clergeau; D., Contardo; M., French; A., Giraldo; G., Guillot; G., Hall; R., Hammarström; R., Haroutunian; L., Jones; T., Kachelhoffer; D., Kryn; M., Loreti; T., K´ladziński; J., Mabo; B., Macevoy; M., Massai; M., Miguet; M., Millmore; A., Morelli; V., Nagaslaev; A., Pallares; F., Parodi; A., Peisert; S., Quian; F., Raffo; M., Raymond; M., Rebouillat; J., Riester; O., Runolfsson; R., Sachdeva; L., Shekhtman; G., Smadja; G., Spandre; M., Spezziga; A., Toropin; C. V., Velde; P., Vanlaer; D., Vit

Extended clinical and immunological phenotype and transplant outcome in CD27 and CD70 deficiency

Biallelic mutations in the genes encoding CD27 or its ligand CD70 underlie inborn errors of immunity (IEIs) characterized predominantly by Epstein-Barr virus (EBV)-associated immune dysregulation, such as chronic viremia, severe infectious mononucleosis, hemophagocytic lymphohistiocytosis (HLH), lymphoproliferation, and malignancy. A comprehensive understanding of the natural history, immune characteristics, and transplant outcomes has remained elusive. Here, in a multi-institutional global collaboration, we collected the clinical information of 49 patients from 29 families (CD27, n = 33; CD70, n = 16), including 24 previously unreported individuals and identified a total of 16 distinct mutations in CD27, and 8 in CD70, respectively. The majority of patients (90%) were EBV1 at diagnosis, but only similar to 30% presented with infectious mononucleosis. Lymphoproliferation and lymphoma were the main clinical manifestations (70% and 43%, respectively), and 9 of the CD27-deficient patients developed HLH. Twenty-one patients (43%) developed autoinflammatory features including uveitis, arthritis, and periodic fever. Detailed immunological characterization revealed aberrant generation of memory B and T cells, including a paucity of EBV-specific T cells, and impaired effector function of CD81 T cells, thereby providing mechanistic insight into cellular defects underpinning the clinical features of disrupted CD27/CD70 signaling. Nineteen patients underwent allogeneic hematopoietic stem cell transplantation (HSCT) prior to adulthood predominantly because of lymphoma, with 95% survival without disease recurrence. Our data highlight the marked predisposition to lymphoma of both CD27- and CD70-deficient patients. The excellent outcome after HSCT supports the timely implementation of this treatment modality particularly in patients presenting with malignant transformation to lymphoma