Giant axonal neuropathy (GAN) is a disorder that affects both the peripheral and central nervous system with clinical and genetic heterogeneity. We describe the spectrum of magnetic resonance imaging (MRI), MR-spectroscopy (MRS), and diffusion changes in two siblings from India. In addition to the described MRI findings two unreported findings, hyperintensity signals of both the globus pallidus...

Giant axonal neuropathy (GAN) is a rare disease caused by mutations in the GAN gene, which encodes gigaxonin, an E3 ligase adapter that targets intermediate filament (IF) proteins for degradation in numerous cell types, including neurons and fibroblasts. The cellular hallmark of GAN pathology is the formation of large aggregates and bundles of IFs. In this study, we show that both the distribut...

Peripheral neuropathy is perhaps the archetypal disease of axonal degeneration, characteristically involving degeneration of the longest axons in the body. Evidence from both inherited and acquired forms of peripheral neuropathy strongly supports that the primary pathology is in the axons themselves and points to disruption of axonal transport as an important disease mechanism. Recent studies i...

Kikkawa T, Takashima A. Practice of gait training using lower-limb orthosis and body weight-supported walker for severe acute motor axonal neuropathy: a case report. Jpn J Compr Rehabil Sci 2023; 14: 49-53.

Magnetic resonance imaging of a girl with giant axonal neuropathy revealed a progressive white matter disease. In close agreement with histopathological features reported previously, localized proton magnetic resonance spectroscopy at 9 and 12 years of age indicated a specific damage or loss of axons (reduced N-acetylaspartate and N-acetylaspartylglutamate) accompanied by acute demyelination (e...

The conical air-void structure of an InGaN light-emitting diode (LEDs) was formed at the GaN/sapphire interface to increase the light extraction efficiency. The fabrication process of the conical air-void structure consisted of a dry process and a crystallographic wet etching process on an undoped GaN layer, followed by a re-growth process for the InGaN LED structure. A higher light output powe...

This study reports on the use of a template that is made of silver nanoparticles (ANPs) that are dispersed on a patterned sapphire substrate (PSS) to improve the light output power of GaN-based light-emitting diodes (LEDs). The dipping of a sapphire substrate in hot H2SO4 solution generates white reaction products that are identified as a mixture of polycrystalline aluminum sulfates. These whit...

J. R. Kirtley,1 B. Kalisky,1,2 J. A. Bert,3 C. Bell,3 M. Kim,3 Y. Hikita,3 H. Y. Hwang,3 J. H. Ngai,4 Y. Segal,4,* F. J. Walker,4 C. H. Ahn,4 and K. A. Moler1,3 1Departments of Physics and Applied Physics, Stanford University, Stanford, California 94305, USA 2Department of Physics, Nano-magnetism Research Center, Institute of Nanotechnology and Advanced Materials, Bar-Ilan University, Ramat-Gan...