The Change of Grip Strength in a Patient with Congenital Myotonic Dystrophy Over a 4-year Period

Myotonic dystrophy (MyD) is a neuromuscular disease that is autosomal dominant and the most common form of muscular dystrophy affecting adults. The clinical features of MyD include a multisystemic disorder characterized by myotonia, progressive muscle weakness and wasting, cataracts, premature balding and mental retardation. The most severe type of MyD is classified as congenital MyD (CMyD). The muscle weakness in CMyD is very severe, but muscle development can be observed in the period of growth. However, no clinical case of this type has been reported yet. Therefore, we report on a girl with CMyD who had an increase in muscle strength over a four-year period. The girl with CMyD participated in this study from the age of 9 to the age of 12. The measurement of muscle strength was recorded as the maximum score of grip strength with the use of dynamometers. Grip strength was assessed once a year by the same two physical therapists. Grip strength of CMyD for each year was markedly weak when compared with the normal controls, but muscle strength changed within some specific growth areas. The muscle weakness in CMyD was remarkable, but the result showed that specific muscle strength of CMyD in childhood was actually increased.     

Synthesis of the P-cluster inorganic core of nitrogenases

The [8Fe-7S] core of the P-clusters in nitrogenases is unique among the known [Fe-S] clusters which are essential to electron-transfer processes in nature. The [8Fe-7S] cluster has been thought unstable and to exist only in protein environments. We found that this unusual [8Fe-7S] structure can be self-assembled from the reaction of Fe(II) bis-amide, tetramethylthiourea, 2,4,6-triisopropylbenzenethiol, and elemental sulfur in a specific mole ratio. The structure of the complex isolated therefrom closely resembles that of the reduced form (PN) of the P-clusters, while the 6Fe(II)2Fe(III) oxidation state was manifested by the M?ssbauer study.     

Organic high-spin systems: Synthesis,electrochemical and spectroscopic studies of π-extended tetraaryl m-phenylenediamines

Three highly π-extended tetraaryl-1,3-phenylenediamines for charged high-spin molecular systems were successfully synthesized using Suzuki coupling. Their electrochemical and spectroscopic properties were also examined. They showed blue fluorescence. The dications derived from highly π-extended tetraaryl-1,3-phenylenediamines were generated and characterized by electron transfer stopped-flow method.