Ulnar dimelia variant: a case report

We report a case of ulnar dimelia, commonly called mirror hand, in a 2-month-old female child who had restriction of elbow flexion and forearm rotation. There was no facial or other internal organ malformation. Radiographs revealed seven triphalangeal digits with double ulnae (one following the other) and absent radius. To the best of the authors’ knowledge, this is the first report of this mirror hand deformity in which fingers are symmetrical while duplicated ulnae are not

Testing foundations of quantum mechanics with photons

The foundational ideas of quantum mechanics continue to give rise to counterintuitive theories and physical effects that are in conflict with a classical description of Nature. Experiments with light at the single photon level have historically been at the forefront of tests of fundamental quantum theory and new developments in photonics engineering continue to enable new experiments. Here we review recent photonic experiments to test two foundational themes in quantum mechanics: wave-particle duality, central to recent complementarity and delayed-choice experiments; and Bell nonlocality where recent theoretical and technological advances have allowed all controversial loopholes to be separately addressed in different photonics experiments.Comment: 10 pages, 5 figures, published as a Nature Physics Insight review articl

The selective post-translational processing of transcription factor Nrf1 yields distinct isoforms that dictate its ability to differentially regulate gene expression

Upon translation, the N-terminal homology box 1 (NHB1) signal anchor sequence of Nrf1 integrates it within the endoplasmic reticulum (ER) whilst its transactivation domains [TADs, including acidic domain 1 (AD1), the flanking Asn/Ser/Thr-rich (NST) domain and AD2] are transiently translocated into the ER lumen, whereupon the NST domain is glycosylated to yield an inactive 120-kDa glycoprotein. Subsequently, these TADs are retrotranslocated into extra-luminal subcellular compartments, where Nrf1 is deglycosylated to yield an active 95-kDa isoform. Herein, we report that AD1 and AD2 are required for the stability of the 120-kDa Nrf1 glycoprotein, but not that of the non-glycosylated/de-glycosylated 95-kDa isoform. Degrons within AD1 do not promote proteolytic degradation of the 120-kDa Nrf1 glycoprotein. However, repositioning of AD2-adjoining degrons (i.e. DSGLS-containing SDS1 and PEST2 sequences) into the cyto/nucleoplasm enables selective topovectorial processing of Nrf1 by the proteasome and/or calpains to generate a cleaved active 85-kDa Nrf1 or a dominant-negative 36-kDa Nrf1γ. Production of Nrf1γ is abolished by removal of SDS1 or PEST2 degrons, whereas production of the cleaved 85-kDa Nrf1 is blocked by deletion of the ER luminal-anchoring NHB2 sequence (aa 81–106). Importantly, Nrf1 activity is positively and/or negatively regulated by distinct doses of proteasome and calpain inhibitors

STUDI EKSPERIMENTAL KARAKTERISTIK KUAT TEKAN DAN KARAKTERISTIK PEMBAKARAN BRIKET DAUN CENGKEH DAN JERAMI PADI

Penelitian ini mempelajari tentang karakteristik kuat tekan dan karakteristik pembakaran briket daun cengkeh dan jerami padi. Pembriketan dilakukan dengan menggunakan mesin pres hidrolik dengan tekanan pembriketan sebesar 450 kg/cm2, dengan bahan pengikat dan tanpa bahan pengikat. Bahan pengikat yang digunakan adalah lem kanji dengan kadar 5 %. Briket berbentuk silinder dengan diameter sekitar 3 cm dan tinggi 5 cm. Variasi parameter pembriketan yang digunakan adalah ukuran butir 20, 40 dan 80 mesh, kadar air 15 %, 20 % dan 25 %, serta suhu pembriketan sebesar 60 oC, 80 oC, 100 oC dan 120 oC. Uji pembakaran dilakukan dalam tungku berbentuk tabung horisontal berdiameter dalam 170 mm. Variasi perameter uji pembakaran yang digunakan adalah kecepatan aliran udara sebesar 0,6 m/s; 0,8 m/s; 1,0 m/s dan 1,2 m/s serta variasi ukuran butir sebesar 20, 40, dan 80 mesh. Suhu pembriketan berpengaruh signifikan terhadap peningkatan kuat tekan briket. Dari hasil uji pembakaran dapat ditentukan besarnya laju pembakaran, profil suhu pembakaran, nilai energi aktivasi (E ), konstanta Arrhenius (A), dan emisi CO. Dari semua percobaan, kadar emisi CO puncak lebih dari 400 ppm. Kata kunci: kuat tekan, daun cengkeh, jerami, bahan pengikat, ukuran butir, suhu pembriketan, kadar air, laju pembakaran, energi aktivasi, emisi CO.

Safety and Feasibility of Long-term Intravenous Sodium Nitrite Infusion in Healthy Volunteers

BACKGROUND: Infusion of sodium nitrite could provide sustained therapeutic concentrations of nitric oxide (NO) for the treatment of a variety of vascular disorders. The study was developed to determine the safety and feasibility of prolonged sodium nitrite infusion. METHODOLOGY: Healthy volunteers, aged 21 to 60 years old, were candidates for the study performed at the National Institutes of Health (NIH; protocol 05-N-0075) between July 2007 and August 2008. All subjects provided written consent to participate. Twelve subjects (5 males, 7 females; mean age, 38.8±9.2 years (range, 21-56 years)) were intravenously infused with increasing doses of sodium nitrite for 48 hours (starting dose at 4.2 µg/kg/hr; maximal dose of 533.8 µg/kg/hr). Clinical, physiologic and laboratory data before, during and after infusion were analyzed. FINDINGS: The maximal tolerated dose for intravenous infusion of sodium nitrite was 267 µg/kg/hr. Dose limiting toxicity occurred at 446 µg/kg/hr. Toxicity included a transient asymptomatic decrease of mean arterial blood pressure (more than 15 mmHg) and/or an asymptomatic increase of methemoglobin level above 5%. Nitrite, nitrate, S-nitrosothiols concentrations in plasma and whole blood increased in all subjects and returned to preinfusion baseline values within 12 hours after cessation of the infusion. The mean half-life of nitrite estimated at maximal tolerated dose was 45.3 minutes for plasma and 51.4 minutes for whole blood. CONCLUSION: Sodium nitrite can be safely infused intravenously at defined concentrations for prolonged intervals. These results should be valuable for developing studies to investigate new NO treatment paradigms for a variety of clinical disorders, including cerebral vasospasm after subarachnoid hemorrhage, and ischemia of the heart, liver, kidney and brain, as well as organ transplants, blood-brain barrier modulation and pulmonary hypertension. CLINICAL TRIAL REGISTRATION INFORMATION: http://www.clinicaltrials.gov; NCT00103025