Limb-girdle muscular dystrophy R9 (LGMDR9) is an autosomal recessive disorder caused by mutations in the fukutin-related protein gene (FKRP), encoding a glycosyltransferase involved in α-dystroglycan modification. Muscle atrophy, a significant feature of LGMDR9, occurs by a change in the normal balance between protein synthesis and protein degradation. The ubiquitin-proteasome system (UPS) and autophagy-lysosomal system play a key role in protein degradation in skeletal muscle cells, but their involvement in the pathology of LGMDR9 is still largely unknown. We have aimed at clarifying whether proteolysis through the UPS and the autophagy-lysosomal pathway is dysregulated in LGMDR9 patients.
 
  Vastus lateralis biopsies from 8 normal controls and 12 LGMDR9 patients harbouring the c.826C>A/c.826C>A FKRP genotype were assessed for protein markers related to UPS, the autophagy-lysosomal system and endoplasmic reticulum (ER) stress/unfolded protein response (UPR), followed by ultrastructural analysis by transmission electron microscopy (TEM).
 
  Protein levels of E3 ubiquitin ligases Atrogin-1 and MuRF1 showed a pattern similar to normal controls. Elevation of the autophagy markers Atg7, LC3B-II, decreased level of p62 as well as downregulation of the negative autophagy regulator mTORC1, indicated an activation of autophagy in LGMDR9. Mitophagy markers Bnip3 and Parkin were decreased. TEM analysis demonstrated accumulation of autophagosome-like structures in LGMDR9 muscle. There was also an increase in the expression of ER stress/UPR markers PDI, peIF2α and CHOP and a decrease in IRE1α. However, GRP94, Bip and Calnexin remained unchanged.
 
  Our findings indicate that autophagy and ER stress are induced in LGMDR9 muscle.
 Our findings indicate that autophagy and ER stress are induced in LGMDR9 muscle.
  to demonstrate scientific evidence on incidence and factors associated with contrast-induced nephropathy in patients undergoing percutaneous coronary intervention.
 
  an integrative review carried out in the VHL, PubMed, VHL Regional Portal and SciELO databases, of articles published between 2014 and 2019.
 
  the sample consisted of five original articles, two cohorts, two control cases and a clinical trial. The incidence of contrast-induced nephropathy ranged from 6% to 24%. It stands out among patients with advanced age, male gender, diabetes mellitus, systemic arterial hypertension, volume of contrast infused and osmolarity.  https://www.selleckchem.com/products/lenalidomide-s1029.html  Intravenous hydration, sodium bicarbonate, ascorbic acid and statin were important prophylactic agents.
 
  this study envisioned the main risk factors for contrast-induced nephropathy in patients undergoing percutaneous coronary intervention and elucidated preventive measures that guide multidisciplinary health care aiming at a quality and safe care.
 this study envisioned the main risk factors for contrast-induced nephropathy in patients undergoing percutaneous coronary intervention and elucidated preventive measures that guide multidisciplinary health care aiming at a quality and safe care.
  To describe the clinical characteristics of patients infected with SARS-CoV-2 at Clinique Ngaliema, a public hospital, in Kinshasa, in the Democratic Republic of Congo (DRC).
 
  This retrospective study analyzed medical records including socio-demographics, past medical history, clinical manifestation, comorbidities, laboratory data, treatment and disease outcome of 160 hospitalized COVID-19 patients, with confirmed result of SARS-CoV-2 viral infection.
 
  The median age of patients was 54 years (IQR 38-64), and there was no significant gender difference (51% of male). The most common comorbidities were hypertension (55 [34%]), diabetes (31 [19%]) and obesity (13 [8%]). Fever (93 [58%]), cough (92 [57%]), fatigue (87 [54%]), shortness of breath (72 [45%]) and myalgia (33 [21%]) were the most common symptoms, upon admission. Patients were categorized into mild (92 [57%]), moderate (19 [12%]) and severe (49 [31%]). Severe patients were older and were more likely to have comorbidities, compared to mild ones. Theo identify patients at higher risk of death at an early stage of the illness. Plasmodium spp co-infection was not common in hospitalized COVID-19 patients.Previous studies showed that conduit artery blood flow rapidly increases after even a brief contraction of muscles within the dependent limb. Whether this rapid hyperemia occurs within contracted skeletal muscle in humans has yet to be confirmed, however. We therefore used diffuse correlation spectroscopy (DCS) to characterize the rapid hyperemia and vasodilatory responses within the muscle microvasculature induced by single muscle contractions in humans. Twenty-five healthy male volunteers performed single 1-s isometric handgrips at 20%, 40%, 60% and 80% of maximum voluntary contraction (MVC). DCS probes were placed on the flexor digitorum superficialis muscle, and a skeletal muscle blood flow index (SMBFI) was derived continuously. At the same time, brachial artery blood flow (BABF) responses were measured using Doppler ultrasound. Single muscle contractions evoked rapid, monophasic increases in both SMBFI and BABF that occurred within 3 s after release of contraction. The initial and peak responses increased with increases in contraction intensity and were greater for BABF than for SMBFI at all intensities. BABF reached its peak within 5 to 8 s after the end of contraction. The SMBFI continued to increase after the BABF passed its peak and was decreasing toward the resting level and peaked about 10 to 15 s after completion of the contraction. We conclude that single muscle contractions induce rapid, intensity-dependent hyperemia within the contracted skeletal muscle microvasculature. Moreover, the characteristics of the rapid hyperemia and vasodilatory responses of skeletal muscle microvessels differ from that simultaneously evaluated in the upstream conduit artery.We present a transformative route to obtain mass-producible helical slow-wave structures for operation in beam-wave interaction devices at THz frequencies. The approach relies on guided self-assembly of conductive nanomembranes. Our work coordinates simulations of cold helices (i.e., helices with no electron beam) and hot helices (i.e., helices that interact with an electron beam). The theoretical study determines electromagnetic fields, current distributions, and beam-wave interaction in a parameter space that has not been explored before. These parameters include microscale diameter, pitch, tape width, and nanoscale surface finish. Parametric simulations show that beam-wave interaction devices based on self-assembled and electroplated helices will potentially provide gain-bandwidth products higher than 2 dBTHz at 1 THz. Informed by the simulation results, we fabricate prototype helices for operation as slow-wave structures at THz frequencies, using metal nanomembranes. Single and intertwined double helices, as well as helices with one or two chiralities, are obtained by self-assembly of stressed metal bilayers.