19 research outputs found
Clinical significance of circulating dendritic cells in patients with systemic lupus erythematosus
The dielectric response of phenothiazine‑based glass‑formers with different molecular complexity
We examined a series of structurally related glass-forming liquids in which a phenothiazine-based
tricyclic core (PTZ) was modified by attaching n-alkyl chains of different lengths (n = 4, 8, 10). We
systematically disentangled the impact of chemical structure modification on the intermolecular
organization and molecular dynamics probed by broadband dielectric spectroscopy (BDS). X-ray
diffraction (XRD) patterns evidenced that all PTZ-derivatives are not ‘ordinary’ liquids and form
nanoscale clusters. The chain length has a decisive impact on properties, exerting a plasticizing effect
on the dynamics. Its elongation decreases glass transition temperature with slight impact on fragility.
The increase in the medium-range order was manifested as a broadening of the dielectric loss peak
reflected in the lower value of stretching parameter βKWW. A disagreement with the behavior observed
for non-associating liquids was found as a deviation from the anti-correlation between the value of
βKWW and the relaxation strength of the α-process. Besides, to explain the broadening of loss peak in
PTZ with the longest (decyl) chain a slow Debye process was postulated. In contrast, the sample with
the shortest alkyl chain and a less complex structure with predominant supramolecular assembly
through π–π stacking exhibits no clear Debye-mode fingerprints. The possible reasons are also
discussed
Deficiency in the mRNA export mediator Gle1 impairs Schwann cell development in the zebrafish embryo
GLE1 mutations cause lethal congenital contracture syndrome 1 (LCCS1), a severe autosomal recessive fetal motor neuron disease, and more recently have been associated with amyotrophic lateral sclerosis (ALS). The gene encodes a highly conserved protein with an essential role in mRNA export. The mechanism linking Gle1 function to motor neuron degeneration in humans has not been elucidated, but increasing evidence implicates abnormal RNA processing as a key event in the pathogenesis of several motor neuron diseases. Homozygous gle1−/− mutant zebrafish display various aspects of LCCS, showing severe developmental abnormalities including motor neuron arborization defects and embryonic lethality. A previous gene expression study on spinal cord from LCCS fetuses indicated that oligodendrocyte dysfunction may be an important factor in LCCS. We therefore set out to investigate the development of myelinating glia in gle1−/− mutant zebrafish embryos. While expression of myelin basic protein (mbp) in hindbrain oligodendrocytes appeared relatively normal, our studies revealed a prominent defect in Schwann cell precursor proliferation and differentiation in the posterior lateral line nerve. Other genes mutated in LCCS have important roles in Schwann cell development, thereby suggesting that Schwann cell deficits may be a common factor in LCCS pathogenesis. These findings illustrate the potential importance of glial cells such as myelinating Schwann cells in motor neuron diseases linked to RNA processing defects
Quality of life and satisfaction with life in SLE patients—the importance of clinical manifestations
To assess the correlation between quality of life (QoL) and satisfaction with life (SL) in SLE patients and correlate both with clinical symptoms of the disease. The study was performed in 83 patients. QoL was assessed by Short Form 36, and SL was assessed by the Satisfaction with Life Scale. Clinical manifestations presented at the time of examination were taken into consideration. SLE patients assessed their QoL and SL as rather low. Those with photosensitivity as well as neurological symptoms presented lower QoL in particular domains, while those with renal manifestation of SLE assessed their QoL as higher. Similar observations were made for SL only in relation to neurological symptoms. Moreover, our findings show that although SL is a part of QoL, both these parameters should be distinguished in order to fully assess the state of the patient