Bridging the Gap Between Nanowires and Josephson Junctions: A Superconducting Device Based on Controlled Fluxon Transfer.

The basis for superconducting electronics can broadly be divided between two technologies: the Josephson junction and the superconducting nanowire. While the Josephson junction (JJ) remains the dominant technology due to its high speed and low power dissipation, recently proposed nanowire devices offer improvements such as gain, high fanout, and compatibility with CMOS circuits. Despite these benefits, nanowire-based electronics have largely been limited to binary operations, with devices switching between the superconducting state and a high-impedance resistive state dominated by uncontrolled hotspot dynamics. Unlike the JJ, they cannot increment an output through successive switching and their operation speeds are limited by their slow thermal-reset times. Thus, there is a need for an intermediate device with the interfacing capabilities of a nanowire but a faster, moderated response allowing for modulation of the output. We present a nanowire device based on controlled fluxon transport. We show that the device is capable of responding proportionally to the strength of its input, unlike other nanowire technologies. The device can be operated to produce a multilevel output with distinguishable states, the number of which can be tuned by circuit parameters. Agreement between experimental results and electrothermal circuit simulations demonstrates that the device is classical and may be readily engineered for applications including use as a multilevel memory.

Clinical presentation of Von Hippel Lindau syndrome type 2B associated with VHL p.A149S mutation in a large Turkish family.

Von Hippel Lindau (VHL) syndrome is an autosomal dominant disorder characterized by benign and malignant tumors. This study presents the clinical and genetic features of VHL syndrome in a Turkish family. For the diagnosis of pheochromocytoma-related diseases, 49 family members from three generations were evaluated between March 2008 and January 2013. Family members were examined to identify components of pheochromocytoma-related genetic syndromes through physical examination, laboratory tests, and imaging methods. For the causative mutation, sequence analysis of VHL gene was performed. Nine patients were diagnosed with pheochromocytoma. Lumbal spinal hemangioblastoma and pancreatic neuroendocrine tumor without pheochromocytoma were detected in one patient. In patients with pheochromocytoma, additional tumors, such as retinal angioma, renal cell carcinoma, pancreatic serous cystadenoma, and pancreatic neuroendocrine tumors were detected. All patients were diagnosed as VHL syndrome type 2B. Sequence analysis of VHL gene revealed heterozygous p.A149S mutation in all symptomatic patients and in seven of the asymptomatic family members. This is the first study that identified VHL p.A149S mutation in a Turkish family with VHL syndrome. However, VHL p.A149S mutation was identified in an American family by Atuk et al. (J Clin Endocrinol Metab, 83:117-120, 14) and the family was defined as VHL type 2A. In our study, the family was identified as VHL type 2B. This variability in the phenotypic features suggests that further studies are required to beter assess the genotype-phenotype correlation in such cases.