New data supporting that early diagnosis and treatment are possible and necessary in intracellular cobalamin depletion: the case of transcobalamin II deficiency.

Objectives: Transcobalamin II (TC) promotes the cellular uptake of cobalamin (Cbl) through receptor-mediated endocytosis of the TC-cbl complex in peripheral tissues. TC deficiency is a rare disorder that causes intracellular Cbl depletion. It presents in early infancy with a failure to thrive, diarrhea, anemia, agammaglobulinemia, and pancytopenia.

Galactic Cosmic Rays Throughout the Heliosphere and in the Very Local Interstellar Medium.

We review recent observations and modeling developments on the subject of galactic cosmic rays through the heliosphere and in the Very Local Interstellar Medium, emphasizing knowledge that has accumulated over the past decade. We begin by highlighting key measurements of cosmic-ray spectra by Voyager, PAMELA, and AMS and discuss advances in global models of solar modulation. Next, we survey recent works related to large-scale, long-term spatial and temporal variations of cosmic rays in different regimes of the solar wind.

[Citrullinemia type I with recurrent liver failure in a child].

Citrullinemia type I is an autosomal recessive disorder caused by mutation of the gene expressing ASS1 argininosuccinate synthetase, limiting enzyme of the urea cycle. The classic variants are associated with neonatal/infantile forms that cause hyperammonemia leading to death if treatment is not established. Initial symptoms of disorders of the urea cycle include neurological impairment with mild or moderate liver damage.

Galactic cosmic ray simulation at the NASA Space Radiation Laboratory.

Most accelerator-based space radiation experiments have been performed with single ion beams at fixed energies. However, the space radiation environment consists of a wide variety of ion species with a continuous range of energies. Due to recent developments in beam switching technology implemented at the NASA Space Radiation Laboratory (NSRL) at Brookhaven National Laboratory (BNL), it is now possible to rapidly switch ion species and energies, allowing for the possibility to more realistically simulate the actual radiation environment found in space.