Anion-Responsive π-Conjugated Macrocycles That Form Ordered Structures.

In this study, anion-responsive π-conjugated macrocycles were synthesized to demonstrate anion-binding and ion-pairing properties along with the ordered structures.  Ion-pairing charge-by-charge assembly of a [1+2]-type complex of a macrocycle as a pseudo π-electronic anion and a countercation was revealed by single-crystal X-ray analysis.  Further, two-dimensional (2D) arrays of the macrocycles bearing alkoxy chains, exhibiting anion-driven disordered structures, were constructed on a highly oriented pyrolytic graphite (HOPG) substrate as observed by scanning tunneling microscopy (STM).

Genetic landscape in undiagnosed patients with syndromic hearing loss revealed by whole exome sequencing and phenotype similarity search.

There are hundreds of rare syndromic diseases involving hearing loss, many of which are not targeted for clinical genetic testing. We systematically explored the genetic causes of undiagnosed syndromic hearing loss using a combination of whole exome sequencing (WES) and a phenotype similarity search system called PubCaseFinder. Fifty-five families with syndromic hearing loss of unknown cause were analyzed using WES after prescreening of several deafness genes depending on patient clinical features.

Dominant effect of a single amino acid mutation in the motor domain of myosin VI on hearing in mice.

An unconventional myosin, myosin VI gene (MYO6), contributes to recessive and dominant hearing loss in humans and mice. The Kumamoto shaker/waltzer (ksv) mouse is a model of deafness resulting from a splice-site mutation in Myo6. While ksv/ksv homozygous mice are deaf due to cochlear hair cell stereocilia fusion at the neonatal stage, the hearing phenotypes of ksv/+ heterozygous mice have been less clear.

Protein profile of mouse endolymph suggests a role in controlling cochlear homeostasis.

The cochlea contains two extracellular fluids, perilymph and endolymph. Endolymph exhibits high potential of approximately +80 to +110 mV (depending on species), which sensitizes sensory hair cells. Other properties of this unique fluid remain elusive, owing to its minuscule volume in rodent cochlea.

Retraction: Photo-responsive hole formation in the monolayer membrane wall of a supramolecular nanotube for quick recovery of encapsulated protein.

[This retracts the article DOI: 10.1039/D2NA00035K.].