Ophthalmological and electrophysiological findings in monozygotic twin sisters with phosphomannomutase 2 deficiency (PMM2-CDG) over a period of 37 years.

To describe the evolution of ophthalmological and electrophysiological findings in monozygotic twin sisters with phosphomannomutase 2 deficiency (PMM2-CDG). A clinical ophthalmological examination with visual acuity measurement, fundoscopy and flash electroretinogram (fERG) was performed at the age of 4, 18 and 41 years. Ophthalmic examination in both girls at the age of 4 years showed an alternating convergent squint and a saccadic pursuit, with visual acuity of 6/9 in both eyes (Ffooks symbols test).

Self-assembly of oppositely charged polyelectrolyte block copolymers containing short thermoresponsive blocks.

The assembly of oppositely charged block copolymers, containing small thermoresponsive moieties, was investigated as a function of salt concentration and temperature. Aqueous solutions of poly-[-isopropylacrylamide]--poly[dimethylaminoethyl methacrylate] (NIPAM--DMAEMA) and PNIPAM--poly[acrylic acid]--PNIPAM (NIPAM--AA--NIPAM) were mixed in equal charge stoichiometry, and analysed by light scattering (LS), NMR spectroscopy and small angle X-ray scattering (SAXS). At room temperature, two different micelle morphologies were found at different salt concentrations.

Rapid and Quantitative De--butylation for Poly(acrylic acid) Block Copolymers and Influence on Relaxation of Thermoassociated Transient Networks.

The synthesis of charged polymers often requires the polymerization of protected monomers, followed by a polymer-analogous reaction to the polyelectrolyte product. We present a mild, facile method to cleave -butyl groups from poly(-butyl acrylate) blocks that yields poly(acrylic acid) (pAA) blocks free of traces of the ester. The reaction utilizes a slight excess of HCl in hexafluoroisopropanol (HFIP) at room temperature and runs to completion within 4 h.

Bioinspired Underwater Adhesives by Using the Supramolecular Toolbox.

Nature has developed protein-based adhesives whose underwater performance has attracted much research attention over the last few decades. The adhesive proteins are rich in catechols combined with amphiphilic and ionic features. This combination of features constitutes a supramolecular toolbox, to provide stimuli-responsive processing of the adhesive, to secure strong adhesion to a variety of surfaces, and to control the cohesive properties of the material.

Heparin as a Bundler in a Self-Assembled Fibrous Network of Functionalized Protein-Based Polymers.

Nature shows excellent control over the mechanics of fibrous hydrogels by assembling protein fibers into bundles of well-defined dimensions. Yet, obtaining artificial materials displaying controlled bundling remains a challenge. Here, we developed genetically engineered protein-based polymers functionalized with heparin-binding KRSR domains and show controlled bundling using heparin as a binder.