2366G>T; p.Gly789Val) that was present only in the calf and dam. This confirmed the diagnosis of cEDS and represents the first report of a causal variant for cEDS in cattle and the first COL5A2-related large animal model.Desmin intermediate filaments (IFs) play an important role in maintaining the structural and functional integrity of muscle cells. They connect contractile myofibrils to plasma membrane, nuclei, and mitochondria. Disturbance of their network due to desmin mutations or deficiency leads to an infringement of myofibril organization and to a deterioration of mitochondrial distribution, morphology, and functions. The nature of the interaction of desmin IFs with mitochondria is not clear. To elucidate the possibility that desmin can directly bind to mitochondria, we have undertaken the study of their interaction in vitro. Using desmin mutant Des(Y122L) that forms unit-length filaments (ULFs) but is incapable of forming long filaments and, therefore, could be effectively separated from mitochondria by centrifugation through sucrose gradient, we probed the interaction of recombinant human desmin with mitochondria isolated from rat liver. Our data show that desmin can directly bind to mitochondria, and this binding depends on its N-terminal domain. We have found that mitochondrial cysteine protease can disrupt this interaction by cleavage of desmin at its N-terminus.Central nervous system (CNS) injury, including stroke, spinal cord injury, and traumatic brain injury, causes severe neurological symptoms such as sensory and motor deficits. Currently, there is no effective therapeutic method to restore neurological function because the adult CNS has limited capacity to regenerate after injury. Many efforts have been made to understand the molecular and cellular mechanisms underlying CNS regeneration and to establish novel therapeutic methods based on these mechanisms, with a variety of strategies including cell transplantation, modulation of cell intrinsic molecular mechanisms, and therapeutic targeting of the pathological nature of the extracellular environment in CNS injury. In this review, we will focus on the mechanisms that regulate CNS regeneration, highlighting the history, recent efforts, and questions left unanswered in this field.Sphingolipids (SLs) are critical components of membrane bilayers that play a crucial role in their physico-chemical properties. Ceramide is the prototype and most studied SL due to its role as a second messenger in the regulation of multiple signaling pathways and cellular processes. Ceramide is a heterogeneous lipid entity determined by the length of the fatty acyl chain linked to its carbon backbone sphingosine, which can be generated either by de novo synthesis from serine and palmitoyl-CoA in the endoplasmic reticulum or via sphingomyelin (SM) hydrolysis by sphingomyelinases (SMases). Unlike de novo synthesis, SMase-induced SM hydrolysis represents a rapid and transient mechanism of ceramide generation in specific intracellular sites that accounts for the diverse biological effects of ceramide. Several SMases have been described at the molecular level, which exhibit different pH requirements for activity neutral, acid or alkaline. Among the SMases, the neutral (NSMase) and acid (ASMase) are the best characterized for their contribution to signaling pathways and role in diverse pathologies, including liver diseases. As part of a Special Issue (Phospholipases From Structure to Biological Function), the present invited review summarizes the physiological functions of NSMase and ASMase and their role in chronic and metabolic liver diseases, of which the most relevant is nonalcoholic steatohepatitis and its progression to hepatocellular carcinoma, due to the association with the obesity and type 2 diabetes epidemic. A better understanding of the regulation and role of SMases in liver pathology may offer the opportunity for novel treatments of liver diseases.The NH3-SCR activities and hydrothermal stabilities of five xCu/MCM-22 zeolites with different Cu loadings (x = 2-10 wt%) prepared by incipient wetness impregnation method were systematically investigated. The physicochemical properties of xCu/MCM-22 zeolites were analyzed by XRD, nitrogen physisorption, ICP-AES, SEM, NH3-TPD, UV-vis, H2-TPR and XPS experiments. The Cu species existing in xCu/MCM-22 are mainly isolated Cu2+, CuO x and unreducible copper species. The concentrations of both isolated Cu2+ and CuO x species in xCu/MCM-22 increase with Cu contents, but the increment of CuO x species is more distinct, especially in high Cu loadings (>4 wt%). NH3-SCR experimental results demonstrated that the activity of xCu/MCM-22 is sensitive to Cu content at low Cu loadings (≤4 wt%). When the Cu loading exceeds 4 wt%, the NH3-SCR activity of xCu/MCM-22 is irrelevant to Cu content due to the severe pore blockage effects caused by aggregated CuO x species. Among the five xCu/MCM-22 zeolites, 4Cu/MCM-22 with moderate Cu content has the best NH3-SCR performance, which displays higher than 80% NO x conversions in a wide temperature window (160-430 °C). Furthermore, the hydrothermal aging experiments (xCu/MCM-22 was treated at 750 °C for 10 h under 10% water vapor atmosphere) illustrated that all the xCu/MCM-22 zeolites exhibit high hydrothermal stability in NH3-SCR reactions.The human interest in donkey milk is growing due to its nutritional, functional properties and excellent microbiological quality according to published reports. However, more research needs to be conducted to assess the above variables from various breeds. In the present study, milk samples were collected from 17 Cypriot and six Arcadian healthy Greek donkeys. The microbiological quality, somatic cell counts (SCC), chemical composition analysis, and antimicrobial activity of the samples was assessed. In addition, clustering and identification of the bacterial composition was performed by RAPD-PCR and 16S rDNA sequencing, respectively. The good microbiological quality of the samples as estimated by the total aerobic mesophilic and psychrotrophic counts, which ranged from 2.18 to 2.71 log CFU/mL and from 1.48 to 2.37 log CFU/mL, respectively, was also verified. SCC were below 4.4 log CFU/mL. However, potential pathogenic species of Staphylococcus aureus, Bacillus cereus, and Clostridium spp. DFP00173 inhibitor were enumerated in the milk of both breeds.