SignificanceScientific peer review has been a cornerstone of the scientific method since the 1600s. Debate continues regarding the merits of single-blind review, in which anonymous reviewers know the authors of a paper and their affiliations, compared with double-blind review, in which this information is hidden. We present an experimental study of this question. In computer science, research often appears first or exclusively in peer-reviewed conferences rather than journals. Our study considers full-length submissions to the highly selective 2017 Web Search and Data Mining conference (15.6% acceptance rate). Each submission is simultaneously scored by two single-blind and two double-blind reviewers. Our analysis shows that single-blind reviewing confers a significant advantage to papers with famous authors and authors from high-prestige institutions.

Spinal muscular atrophy (SMA) is a motor neuron disease caused by dysfunction of the survival motor neuron (SMN) gene. Human SMN gene is present in duplicated copies: SMN1 and SMN2. More than 95% of patients with SMA lack a functional SMN1 but retain at least one copy of SMN2. Unlike SMN1, SMN2 is primarily transcribed into truncated messenger RNA and produces low levels of SMN protein. We tested a therapeutic strategy by treating cultured lymphocytes from patients with SMA with hydroxyurea to modify SMN2 gene expression and to increase the production of SMN protein. Twenty lymphoblastoid cell lines (15 SMA and 5 control lines) were treated with hydroxyurea at 5 concentrations (0.5, 5, 50, 500, and 5,000 microg/ml) and 3 time points (24, 48, and 72 hours). SMN2 gene copy numbers were determined using real-time quantitative polymerase chain reaction. Hydroxyurea treatment resulted in a time-related and dose-dependent increase in the ratio of full-length to truncated SMN messenger RNA. SMN protein levels and intranuclear gems also were significantly increased in these hydroxyurea-treated cells. The SMN2 gene copy number correlated inversely with the SMA phenotypic severity. This study provides the first evidence for a therapeutic indication of hydroxyurea in SMA.

No abstract

No abstract

No abstract