Nonsense mutation in the phosphofructokinase muscle subunit gene associated with retention of intron 10 in one of the isolated transcripts in Ashkenazi Jewish patients with Tarui disease.

Mutations in the human phosphofructokinase muscle subunit gene (PFKM) are known to cause myopathy classified as glycogenosis type VII (Tarui disease). Previously described molecular defects include base substitutions altering encoded amino acids or resulting in abnormal splicing. We report a mutation resulting in phosphofructokinase deficiency in three patients from an Ashkenazi Jewish family. Using a reverse transcription PCR assay, PFKM subunit transcripts differing by length were detected in skeletal muscle tissue of all three affected subjects. In the longer transcript, an insertion of 252 nucleotides totally homologous to the structure of the 10th intron of the PFKM gene was found separating exon 10 from exon 11. In addition, two single base transitions were identified by direct sequencing: [exon 6; codon 95; CGA (Arg) to TGA (stop)] and [exon 7; codon 172; ACC (Thr) to ACT (Thr)] in either transcript. Single-stranded conformational polymorphism and restriction enzyme analyses confirmed the presence of these point substitutions in genomic DNA and strongly suggested homozygosity for the pathogenic allele. The nonsense mutation at codon 95 appeared solely responsible for the phenotype in these patients, further expanding genetic heterogeneity of Tarui disease. Transcripts with and without intron 10 arising from identical mutant alleles probably resulted from differential pre-mRNA processing and may represent a novel message from the PFKM gene.

Pipe3D, a pipeline to analyse integral Field Spectroscopy data: I. New fitting philosophy of FIT3D

We present an improved version of FIT3D, a fitting tool for the analysis of the spectroscopic properties of the stellar populations and the ionized gas derived from moderate resolution spectra of galaxies. This tool was developed to analyze integral field spectroscopy data and it is the basis of PIPE3D, a pipeline used in the analysis of CALIFA, MaNGA, and SAMI data. We describe the philosophy and each step of the fitting procedure. We present an extensive set of simulations in order to estimate the precision and accuracy of the derived parameters for the stellar populations and the ionized gas. We report on the results of those simulations. Finally, we compare the results of the analysis using FIT3D with those provided by other widely used packages, and we find that the parameters derived by FIT3D are fully compatible with those derived using these other tools.

The Jewish Law Firm: Past and Present

The rise and growth of large Jewish law firms in New York City during the second half of the twentieth century was nothing short of an astounding success story. As late as 1950, there was not a single large Jewish law firm in town. By the mid-1960s, six of the largest twenty law firms were Jewish, and by 1980, four of the largest ten prestigious law firms were Jewish firms. Moreover, the accomplishment of the Jewish firms is especially striking because, while the traditional large White Anglo-Saxon Protestant law firms grew at a fast rate during this period, the Jewish firms grew twice as fast, and they did so in spite of experiencing explicit discrimination. What happened? This book chapter is a revised, updated study of the rise and growth of large New York City Jewish law firms. It is based on the public record, with respect to both the law firms themselves and trends in the legal profession generally, and on over twenty in-depth interviews with lawyers who either founded and practiced at these successful Jewish firms, attempted and failed to establish such firms, or were in a position to join these firms but decided instead to join WASP firms. According to the informants interviewed in this chapter, while Jewish law firms benefited from general decline in anti-Semitism and increased demand for corporate legal services, a unique combination of factors explains the incredible rise of the Jewish firms. First, white-shoe ethos caused large WASP firms to stay out of undignified practice areas and effectively created pockets of Jewish practice areas, where the Jewish firms encountered little competition for their services. Second, hiring and promotion discriminatory practices by the large WASP firms helped create a large pool of talented Jewish lawyers from which the Jewish firms could easily recruit. Finally, the Jewish firms benefited from a flip side of bias phenomenon, that is, they benefited from the positive consequences of stereotyping. Paradoxically, the very success of the Jewish firms is reflected in their demise by the early twenty-first century: because systematic large law firm ethno-religious discrimination against Jewish lawyers has become a thing of the past, the very reason for the existence of Jewish law firms has been nullified. As other minority groups, however, continue to struggle for equality within the senior ranks of Big Law, can the experience of the Jewish firms serve as a “separate-but-equal” blueprint for overcoming contemporary forms of discrimination for women, racial, and other minority attorneys? Perhaps not. As this chapter establishes, the success of large Jewish law firms was the result of unique conditions and circumstances between 1945 and 1980, which are unlikely to be replicated. For example, large law firms have become hyper-competitive and are not likely to allow any newcomers the benefit of protected pockets of practice. While smaller “separate-but-equal” specialized firms, for instance, ones exclusively hiring lawyer-mothers occasionally appear, the rise of large “separate-but-equal” firms is improbable.

[Natural Philosophy book list, 1790]

One leaf containing a handwritten list of books marked "Natural Philosophy." Entries contain the author's last name and abbreviated title; five books have dates. The page is inscribed "Callender" and likely refers to John Callender (Harvard AB 1790).

[Ethics and Moral Philosophy book list, 1790]

One leaf containing a handwritten list of books with one column marked "Ethics" and the other column marked "Moral Philosophy." Entries contain the author's last name and abbreviated title. The page is inscribed "Vose," likely referring to Roger Vose (Harvard AB 1790), and the verso has a fragment of a poem.

[Ethics and moral philosophy book list, 1790]

One leaf containing a handwritten list of books generally on ethics and moral philosophy, though no subject is identified. Entries contain the author's last name and abbreviated title. The page is inscribed "Paine," likely referring to Robert Treat Paine (Harvard AB 1792), and the verso has a fragment of a student essay on the theme "An undevout astronomer is mad," signed "Paine."

Rules and orders relating to a Professor of the Mathematicks, of Natural and Experimental Philosophy, in Harvard College, 18 January 1726

This folder contains a single document describing the "rules and orders" of the Hollis Professor of Mathematics and Natural Philosophy. The document begins by defining the subjects to be taught by the Hollis Professor including natural and experimental philosophy, elements of geometry, and the principles of astronomy and geography. It then outlines the number of public and private lectures to be given to students, how much extra time the professor should spend with students reviewing any difficulties they may encounter understanding class subject matter discussed, and stipulates that the professor's duties shall be restricted solely to his teaching activities and not involve him in any religious activities at the College or oblige him to teach any additional studies other than those specified for the Hollis Professor of Mathematics and Natural Philosophy. Furthermore, the rules establish the professor's salary at £80 per year and allow the professor to receive from students, except those students studying theology under the Hollis Professor of Divinity, an additional fee as determined by the Corporation and Board of Overseers, to supplement his income. Moreover, the rules assert that all professorship candidates selected by the Harvard Corporation must be approved by Thomas Hollis during his lifetime or by his executor after his death. Finally, the rules state that the Hollis professor take an oath to the civil government and declare himself a member of the Protestant reformed religion. This document is signed by Thomas Hollis and four witnesses, John Hollis, Joshua Hollis, Richard Solly, and John Williams.

Short nomenclator or vocabular in English and Hebrew: composed alphabetically for the use and benefit of my pupils in particular and for the advantage of those who are desirous to obtain the knowledge of the Hebrew tongue in general, which may be that great help to understand not only ye sacred oracles in their original, but even any Jewish author (so far as concern owns) as also it may give great insight in ye tongue to those, as to compose it, a work altogether new, [ca. 1735]

Headed on the first page with the words "Nomenclatura hebraica," this handwritten volume is a vocabulary with the Hebrew word in the left column, and the English translation on the right. While the book is arranged in sections by letter, individual entries do not appear in strict alphabetical order. The small vocabulary varies greatly and includes entries like enigma, excommunication, and martyr, as well as cucumber and maggot. There are translations of the astrological signs at the end of the volume. Poem written at the bottom of the last page in different hand: "Women when good the best of saints/ that bright seraphick lovely/ she, who nothing of an angel/ wants but truth & immortality./ Verse 2: Who silken limbs & charming/ face. Keeps nature warm."

The summary of a course of experimental lectures in natural philosophy by Dr. John Winthrop (manuscript copy), 1772-1774

Notebook with a handwritten copy of lecture summaries for a physics course given by Harvard Professor John Winthrop. The notes were made by Timothy Foster in 1772 and 1773. The volume contains twenty-five lectures with some diagrams.

A synopsis of naturall philosophy according to the method of the ancients, but improved & augmented with the notions of later philosophers : manuscript, 1677-1679

Possibly autograph, dated at end of volume: Finitu[m] mart: 14, 1678/9. Imperfect copy with title page missing; supplied from a MS copy, dated 29 March 1680, now in the Bodleian Library.

Human creation, imagination and autonomy: a brief introduction to Castoriadis’ social and psychoanalytical philosophy

During the last decade Castoriadis’ questioning has become a reference point in contemporary social theory. In this article I examine some of the key notions in Castoriadis’ work and explore how he strives to develop a theory on the irreducible creativity in the radical imagination of the individual and in the institution of the social-historical sphere. Firstly, I briefly discuss his conception of modern capitalism as bureaucratic capitalism, a view initiated by his criticism of the USSR regime. The following break up with Marxist theory and his psychoanalytic interests empowered him to criticize Lacan and read Freud in an imaginative, though unorthodox, fashion. I argue that this critical enterprise assisted greatly Castoriadis in his conception of the radical imaginary and in his unveiling of the political aspects of psychoanalysis. On the issue of the radical imaginary and its methodological repercussions, I’m focusing mainly on the radical imagination of the subject and its importance in the transition from the ‘‘psychic’’ to the ‘‘subject’’. Taking up the notion of “Being” as a starting point, I examine the notion of autonomy, seeking its roots in the ancient Greek world. By looking at notions such as “praxis”, “doing”, “project” and “elucidation”, I show how Castoriadis sought to redefine revolution as a means for social and individual autonomy. Finally I attempt to clarify the meaning of “democracy” and “democratic society” in the context of the social imaginary and its creations, the social imaginary significations.

Gibt es eine politische Philosophie der Europäischen Union? = Is there a political philosophy of the European Union?. ZEI Discussion Paper C223, 2014

In a theoretical context, the European Union is generally interpreted through the prism of integration theories, which in turn reflect the ever changing empirical reality of the integration process. ZEI Director Ludger Kühnhardt asks if and to what extent the process of European integration has begun to generate a specific political philosophy which uses the EU - and not the classical notion of the state – as the starting and reference point for its reasoning. Kühnhardt examines examples – such as the European notion of civil rights and the notion of the Union itself, but also critical categories such as euroskepticism – which indicate that the EU itself is beginning to be the starting point and frame of reference for a reflection on the common good. For now, a political philosophy in the context of the European Union exists only in an embryonic stage, but the topic may generate intellectual insights through further and deeper research.