Memoirs of David Blaustein : educator and communal worker

arranged by Miriam Blaustein

Dr. David Einhorn und seine Bedeutung für das Judentum

Vortrag geh. ... von Adolf Brüll

David Deutsch (1810-73), ein Rabbiner in Myslowitz u. Sohrau O.-S. : ein Lebensbild

von J. Norden. Hrsg. vom "Verein f. jüd. Geschichte u. Litteratur"

Samuel David Luzzatto : ein Gedenkbuch zum 100. Geburtstage, 22. August 1900

hrsg. vom Verband d. Vereine f. Jüdische Geschichte u. Litteratur in Deutschland. Mit Beitr. von W. Bacher [u.a.]

David Kaufmann : eine Biographie

von Samuel Krauß

Geschichte David's und der Königsherrschaft in Israel

Diese Ausg. ist im Freimann-Kat. nicht enthalten; Seiten auch in der Druckvorlage teilw. in schlechter Qualität

Catalogue de la bibliothèque de littérature hébra͏̈ique et orientale de feu Samuel David Luzzatto de Trieste

par son fils Joseph

A large animal model of spinal muscular atrophy and correction of phenotype.

OBJECTIVES Spinal muscular atrophy (SMA) is caused by reduced levels of survival motor neuron (SMN) protein, which results in motoneuron loss. Therapeutic strategies to increase SMN levels including drug compounds, antisense oligonucleotides, and scAAV9 gene therapy have proved effective in mice. We wished to determine whether reduction of SMN in postnatal motoneurons resulted in SMA in a large animal model, whether SMA could be corrected after development of muscle weakness, and the response of clinically relevant biomarkers. METHODS Using intrathecal delivery of scAAV9 expressing an shRNA targeting pig SMN1, SMN was knocked down in motoneurons postnatally to SMA levels. This resulted in an SMA phenotype representing the first large animal model of SMA. Restoration of SMN was performed at different time points with scAAV9 expressing human SMN (scAAV9-SMN), and electrophysiology measurements and pathology were performed. RESULTS Knockdown of SMN in postnatal motoneurons results in overt proximal weakness, fibrillations on electromyography indicating active denervation, and reduced compound muscle action potential (CMAP) and motor unit number estimation (MUNE), as in human SMA. Neuropathology showed loss of motoneurons and motor axons. Presymptomatic delivery of scAAV9-SMN prevented SMA symptoms, indicating that all changes are SMN dependent. Delivery of scAAV9-SMN after symptom onset had a marked impact on phenotype, electrophysiological measures, and pathology. INTERPRETATION High SMN levels are critical in postnatal motoneurons, and reduction of SMN results in an SMA phenotype that is SMN dependent. Importantly, clinically relevant biomarkers including CMAP and MUNE are responsive to SMN restoration, and abrogation of phenotype can be achieved even after symptom onset.

Same same but different: Locational policies in small and medium-sized European cities

Globalized interurban competition is affecting cities of various sizes and locations. Small and medium-sized cities have to find ways to position themselves in global markets by formulating locational policies. This paper outlines an analytical framework of locational policies that cities adopt in order to increase their competitiveness. By comparing two European small and mediumsized cities (Lucerne and Ulm), we examine manifestations of locational policies and compare if these policies are being diverse or resemble each other. We found that strategies of both cities are sharing the intentions to be competitive, but their policy choices differ because the economic and political context is enabling or restricting certain kinds of locational policies. Furthermore, the findings point to the high explanatory power of municipal tax autonomy when studying locational policies.