During a study of hereditary motor and sensory neuropathy-Lom in Bulgaria, a previously unrecognized neurological disorder was encountered, mainly in Wallachian Gypsies, who represent a relatively recent genetic isolate. The disorder has been termed the congenital cataracts facial dysmorphism neuropathy (CCFDN) syndrome to emphasize its salient features. Fifty individuals from 19 extended pedigrees were identified and examined clinically and electrophysiologically. At least 1 patient from each family was admitted to the hospital in Sofia for full investigation. Pedigree analysis indicates autosomal recessive inheritance. The disorder is recognized in infancy by the presence of congenital cataracts and microcorneas. A predominantly motor neuropathy beginning in the lower limbs and later affecting the upper limbs develops during childhood and leads to severe disability by the third decade. Associated neurological features are a moderate nonprogressive cognitive deficit in most affected individuals together with pyramidal signs and mild chorea in some. Accompanying nonneurological features include short stature, characteristic facial dysmorphism, and hypogonadotrophic hypogonadism. Nerve conduction studies suggest a hypomyelinating/demyelinating neuropathy, confirmed by nerve biopsy. The CCFDN syndrome is thus a pleomorphic autosomal recessive disorder displaying a combination of neurological and nonneurological features.
Nickel is one of the most dangerous environmental pollutants and its removal from wastewater is an important task. The capacity of a mineral–organic hybrid adsorbent, consisting of Shewanella xiamenensis biofilm and zeolite (clinoptilolite of the Chola deposit), to remove metal ions from nickel-containing batch systems under different experimental conditions was tested. The obtained biosorbent was characterized using neutron activation, SEM, and FTIR techniques. It was established that maximum removal of cations, up to 100%, was achieved at pH 6.0. Several mathematical models were applied to describe the equilibrium and kinetics data. The maximum adsorption capacity of the hybrid biosorbent, calculated using the Langmuir model, varied from 3.6 to 3.9 mg/g. Negative Gibbs energy values and positive ∆H° values indicate the spontaneous and endothermic character of the biosorption process. The effects of several parameters (pH and biosorbent dosage) on Ni(II) removal from real effluent, containing nickel with a concentration of 125 mg/L, were investigated. The optimal pH for Ni(II) removal was 5.0–6.0 and an increase of sorbent dosage from 0.5 to 2.0 led to an increase in Ni(II) removal from 17% to 27%. At two times effluent dilution, maximum Ni(II) removal of 26% was attained at pH 6.0 and sorbent dosage of 1.0 g. A 12-fold effluent dilution resulted in the removal of 72% of Ni(II) at the same pH and sorbent dosage values. The obtained hybrid biosorbent can be used for Ni(II) removal from industrial effluents with low Ni(II) concentrations.
The development of different methods for tuning surface properties is currently of great interest. The presented work is devoted to the use of modified nanodiamonds to control the wetting and biological fouling of polymers using optical sensors as an example. We have shown that, depending on the type of modification and the amount of nanodiamonds, the surface of the same fluorinated polymer can have both bactericidal properties and, on the contrary, good adhesion to the biomaterial. The precise control of wetting and biofouling properties of the surface was achieved by the optimization of the modified nanodiamonds thermal anchoring conditions. In vitro and in vivo tests have shown that the fixation of amine functional groups leads to inhibition of biological activity, while the presence of a large number of polar groups of mixed composition (amide and acid chloride) promotes adhesion of the biomaterial and allows one to create a biosensor on-site. A comprehensive study made it possible to establish that in the first 5 days the observed biosensor response is provided by cells adhered to the surface due to the cell wall interaction. On the 7th day, the cells are fixed by means of the polysaccharide matrix, which provides much better retention on the surface and a noticeably greater response to substrate injections. Nevertheless, it is important to note that even 1.5 h of incubation is sufficient for the formation of the reliable bioreceptor on the surface with the modified nanodiamonds. The approach demonstrated in this work makes it possible to easily and quickly isolate the microbiome on the surface of the sensor and perform the necessary studies of its substrate specificity or resistance to toxic effects.
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