Clinical and biochemical improvements in a patient with MNGIE following enzyme replacement.

Mitochondrial neurogastrointestinal encephalomyopathy (MNGIE) is a rare autosomal recessive metabolic disorder caused by a deficiency of thymidine phosphorylase (TP, EC2.4.2.4) due to mutations in the nuclear gene TYMP. TP deficiency leads to plasma and tissue accumulations of thymidine and deoxyuridine which generate imbalances within the mitochondrial nucleotide pools, ultimately leading to mitochondrial dysfunction.1 MNGIE is characterized clinically by leukoencephalopathy, external ophthalmoplegia, peripheral polyneuropathy, cachexia, and enteric neuromyopathy manifesting as gastrointestinal dysmotility. The condition is relentlessly progressive, with patients usually dying from a combination of nutritional and neuromuscular failure at an average age of 37 years.2 Allogeneic hematopoietic stem cell transplantation (AHSCT) offers a permanent cure. Clinical and biochemical improvements following AHSCT have been reported but it carries a high mortality risk and is limited by matched donor availability.3 A consensus proposal for standardizing AHSCT recommends treatment of patients without irreversible end-stage disease and with an optimally matched donor; a majority of patients are ineligible and thus there is a critical requirement for an alternative treatment

Potential of laser for SPS power transmission

Research on the feasibility of using a laser subsystem as an additional option for the transmission of the satellite power system (STS) power is presented. Current laser work and predictions for future laser performance provide a level of confidence that the development of a laser power transmission system is technologically feasible in the time frame required to develop the SBS. There are significant economic advantages in lower ground distribution costs and a reduction of more than two orders of magnitude in real estate requirements for ground based receiving/conversion sites

Cu-Au type orderings in the staggered quadrupolar region of the fcc Blume Emery Griffiths model

The spin-1 Ising (BEG) model has been simulated using a cellular automaton (CA) algorithm improved from the Creutz cellular automaton (CCA) for a face-centered cubic (fcc) lattice. The ground state diagram ($k$, $d$) of the fcc BEG model has ferromagnetic ($F$), quadrupolar ($Q$) and staggered quadrupolar ($SQ$) ordering regions. The simulations have been made in the staggered quadrupolar region for the parameter values in the intervals $-24\leq d=D/J<0$ and $-3\leq k=K/J\leq 0$ . The phase diagrams on the ($kT_{C}/J$, $d$) and the ($kT_{C}/J$, $k$) planes have been obtained through $k=-3$ and $d=-4$ lines, respectively. The staggered quadrupolar ordering region separates into five ordering regions ($A_{3}B(a)$, $A_{3}B(f)$, $AB$ (type-I), $AB$(type-II) and $AB_{3}(f)$) which have the different stoichiometric Cu-Au type structures.Comment: 24 pages, 11 figure

Satellite Power System (SPS) military implications

The military implications of the reference satellite power system (SPS) were examined is well as important military related study tasks. Primary areas of investigation were the potential of the SPS as a weapon, for supporting U.S. military preparedness, and for affecting international relations. In addition, the SPS's relative vulnerability to overt military action, terrorist attacks, and sabotage was considered

Cost effective flat plate photovoltaic modules using light trapping

Work in optical trapping in 'thick films' is described to form a design guide for photovoltaic engineers. A thick optical film can trap light by diffusive reflection and total internal reflection. Light can be propagated reasonably long distances compared with layer thicknesses by this technique. This makes it possible to conduct light from inter-cell and intra-cell areas now not used in photovoltaic modules onto active cell areas