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Reversible Hydrogen Storage via Titanium-Catalyzed LiAlH 4 and Li 3 AlH 6 .-Nanocrystalline powders of (TiCl 3 ·1/3AlCl 3 )-doped LiAlH 4 and Li 3 AlH 6 are prepared using a vibrating-mill technique, which can activate the reaction system by bringing the reagents into very close contact at the preparative scale and by providing extra mechanical energy, much more effectively than the well-known ball-milling method. The samples are characterized by powder XRD, SEM, TG, and DSC. As revealed by XPS and thermodynamic and kinetic measurements the Ti-doped LiAlH 4 and Li 3 AlH 6 samples exhibit good characteristics in terms of reactivity in dehydriding/rehydriding reactions, suggesting their attractive application as reversible hydrogen storage materials.

Friedreich ataxia (FRDA), the most common autosomal recessive neurodegenerative disease among Europeans and people of European descent, is characterized by an early onset (usually before the age of 25), progressive ataxia, sensory loss, absence of tendon reflexes and pyramidal weakness of the legs. We have recently identified a unique group of patients whose clinical presentations are characterized by autosomal recessive inheritance, early age of onset, FRDA-like clinical presentations and hypoalbuminemia. Linkage to the FRDA locus, however, was excluded. Given the similarities of the clinical presentations to those of the recently described ataxia with oculomotor apraxia (AOA) linked to chromosome 9p13, we confirmed that the disorder of our patients is also linked to the same locus. We narrowed the candidate region and have identified a new gene encoding a member of the histidine triad (HIT) superfamily as the 'causative' gene. We have called its product aprataxin; the gene symbol is APTX. Although many HIT proteins have been identified, aprataxin is the first to be linked to a distinct phenotype.

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