Correlating Infall with Deuterium Fractionation in Dense Cores

We present a survey of HCO+ (3-2) observations pointed towards dense cores with previous measurements of N(N2D+)/N(N2H+). Of the 26 cores in this survey, five show the spectroscopic signature of outward motion, nine exhibit neither inward nor outward motion, eleven appear to be infalling, and one is not detected. We compare the degree of deuterium fractionation with infall velocities calculated from the HCO+ spectra and find that those cores with [D]/[H] > 0.1 are more likely to have the signature of inward motions than cores with smaller [D]/[H] ratios. Infall motions are also much more common in cores with masses exceeding their thermal Jeans masses. The fastest infall velocity measured belongs to one of the two protostellar cores in our survey, L1521F, and the observed motions are typically on the order of the sound speed.Comment: Accepted to Ap

Highly turbulent gas on GMC-scales in NGC 3256, the nearest luminous infrared galaxy

We present the highest resolution CO (2-1) observations obtained to date (0.25") of NGC 3256 and use them to determine the detailed properties of the molecular interstellar medium in the central 6 kpc of this merger. Distributions of physical quantities are reported from pixel-by-pixel measurements at 55 and 120 pc scales and compared to disc galaxies observed by PHANGS-ALMA. Mass surface densities range from 8 to 5500 M$_{\odot}$ pc$^{-2}$ and velocity dispersions from 10 to 200 km s$^{-1}$. Peak brightness temperatures as large as 37 K are measured, indicating the gas in NGC 3256 may be hotter than all regions in nearby disc galaxies measured by PHANGS-ALMA. Brightness temperatures even surpass those in the overlap region of NGC 4038/9 at the same scales. The majority of the gas appears unbound with median virial parameters of 7 to 19, although external pressure may bind some of the gas. High internal turbulent pressures of 10$^{5}$ to 10$^{10}$ K cm$^{-3}$ are found. Given the lack of significant trends in surface density, brightness temperature, and velocity dispersion with physical scale we argue the molecular gas is made up of a smooth medium down to 55 pc scales, unlike the more structured medium found in the PHANGS-ALMA disc galaxies.Comment: accepted to MNRAS, 21 pages, 8 figure

Requirement of argininosuccinate lyase for systemic nitric oxide production

Nitric oxide (NO) is crucial in diverse physiological and pathological processes. We show that a hypomorphic mouse model of argininosuccinate lyase (encoded by Asl) deficiency has a distinct phenotype of multiorgan dysfunction and NO deficiency. Loss of Asl in both humans and mice leads to reduced NO synthesis, owing to both decreased endogenous arginine synthesis and an impaired ability to use extracellular arginine for NO production. Administration of nitrite, which can be converted into NO in vivo, rescued the manifestations of NO deficiency in hypomorphic Asl mice, and a nitric oxide synthase (NOS)-independent NO donor restored NO-dependent vascular reactivity in humans with ASL deficiency. Mechanistic studies showed that ASL has a structural function in addition to its catalytic activity, by which it contributes to the formation of a multiprotein complex required for NO production. Our data demonstrate a previously unappreciated role for ASL in NOS function and NO homeostasis. Hence, ASL may serve as a target for manipulating NO production in experimental models, as well as for the treatment of NO-related diseases

The historical origins of corruption in the developing world: a comparative analysis of East Asia

A new approach has emerged in the literature on corruption in the developing world that breaks with the assumption that corruption is driven by individualistic self-interest and, instead, conceptualizes corruption as an informal system of norms and practices. While this emerging neo-institutionalist approach has done much to further our understanding of corruption in the developing world, one key question has received relatively little attention: how do we explain differences in the institutionalization of corruption between developing countries? The paper here addresses this question through a systematic comparison of seven developing and newly industrialized countries in East Asia. The argument that emerges through this analysis is that historical sequencing mattered: countries in which the "political marketplace" had gone through a process of concentration before universal suffrage was introduced are now marked by less harmful types of corruption than countries where mass voting rights where rolled out in a context of fragmented political marketplaces. The paper concludes by demonstrating that this argument can be generalized to the developing world as a whole

Property and Contract Rights in Autocracies and Democracies

We present and test empirically a new theory of property and contract rights. Any incentive an autocrat has to respect such rights comes from his interest in future tax collections and national income and increases with his planning horizon. We find a compelling empirical relationship between property and contract rights and an autocrat's time in power. In lasting -- but not in new -- democracies, the same rule of law and individual rights that ensure continued free elections entail extensive property and contract rights. We show that the age of a democratic system is strongly correlated with property and contract rights

Association of Variants in the SPTLC1 Gene With Juvenile Amyotrophic Lateral Sclerosis

Importance: Juvenile amyotrophic lateral sclerosis (ALS) is a rare form of ALS characterized by age of symptom onset less than 25 years and a variable presentation.Objective: To identify the genetic variants associated with juvenile ALS.Design, Setting, and Participants: In this multicenter family-based genetic study, trio whole-exome sequencing was performed to identify the disease-associated gene in a case series of unrelated patients diagnosed with juvenile ALS and severe growth retardation. The patients and their family members were enrolled at academic hospitals and a government research facility between March 1, 2016, and March 13, 2020, and were observed until October 1, 2020. Whole-exome sequencing was also performed in a series of patients with juvenile ALS. A total of 66 patients with juvenile ALS and 6258 adult patients with ALS participated in the study. Patients were selected for the study based on their diagnosis, and all eligible participants were enrolled in the study. None of the participants had a family history of neurological disorders, suggesting de novo variants as the underlying genetic mechanism.Main Outcomes and Measures: De novo variants present only in the index case and not in unaffected family members.Results: Trio whole-exome sequencing was performed in 3 patients diagnosed with juvenile ALS and their parents. An additional 63 patients with juvenile ALS and 6258 adult patients with ALS were subsequently screened for variants in the SPTLC1 gene. De novo variants in SPTLC1 (p.Ala20Ser in 2 patients and p.Ser331Tyr in 1 patient) were identified in 3 unrelated patients diagnosed with juvenile ALS and failure to thrive. A fourth variant (p.Leu39del) was identified in a patient with juvenile ALS where parental DNA was unavailable. Variants in this gene have been previously shown to be associated with autosomal-dominant hereditary sensory autonomic neuropathy, type 1A, by disrupting an essential enzyme complex in the sphingolipid synthesis pathway.Conclusions and Relevance: These data broaden the phenotype associated with SPTLC1 and suggest that patients presenting with juvenile ALS should be screened for variants in this gene.</p