Tay-Sachs disease is a prototypic neurodegenerative disease. Lysosomal storage of GM2 ganglioside in Tay-Sachs and the related disorder, Sandhoff disease, is caused by deficiency of beta-hexosaminidase A, a heterodimeric protein. Tay-Sachs-related diseases (GM2 gangliosidoses) are incurable, but gene therapy has the potential for widespread correction of the underlying lysosomal defect by means...

The introduction in recent years of more refined techniques for the isolation and characterization of biological lipids has led to the detection of a number of disorders which share with Tay-Sachs disease a disturbance of ganglioside metabolism. Gangliosides are sphingosine-containing glycolipids distinguished by the inclusion of neuraminic acid, and the various gangliosidoses are attributable ...

INTRODUCTION The atlas by Heinrich Sachs (1892) provided an accurate description of the intralobar fibres of the occipital lobe, with a detailed representation of the short associative tracts connecting different parts of the lobe. Little attention has been paid to the work of Sachs since its publication. In this study, we present the results of the dissection of three hemispheres, performed ac...

We present new exact solutions of the low-energy-effective-action string equations with both dilaton φ and axion H fields non-zero. The background universe is of Kantowski-Sachs type. We consider the possibility of a pseudoscalar axion field h (H = eφ(dh)∗) that can be either time or space dependent. The case of time-dependent h reduces to that of a stiff perfect-fluid cosmology. For space-depe...

This is the first complete translation of Heinrich Sachs' outstanding white matter atlas dedicated to the occipital lobe. This work is accompanied by a prologue by Prof Carl Wernicke who for many years was Sachs' mentor in Breslau and enthusiastically supported his work.

conclusions glenohumeral joint reduction with reconstruction of the humeral head seems to be possible even in a neglected locked posterior shoulder fracture-dislocation. if the depressed osteochondral fragment is still spheric, signs of the avascular necrosis are absent on ct, and mal:union: of the fragment has occurred, it is quite possible to reconstruct the head. although results of the case...