scholarly journals Mechanisms of Neuronal Damage in Acute Hepatic Porphyrias

Diagnostics ◽  
2021 ◽  
Vol 11 (12) ◽  
pp. 2205
Author(s):  
Andrea Ricci ◽  
Elena Di Pierro ◽  
Matteo Marcacci ◽  
Paolo Ventura

Porphyrias are a group of congenital and acquired diseases caused by an enzymatic impairment in the biosynthesis of heme. Depending on the specific enzyme involved, different types of porphyrias (i.e., chronic vs. acute, cutaneous vs. neurovisceral, hepatic vs. erythropoietic) are described, with different clinical presentations. Acute hepatic porphyrias (AHPs) are characterized by life-threatening acute neuro-visceral crises (acute porphyric attacks, APAs), featuring a wide range of neuropathic (central, peripheral, autonomic) manifestations. APAs are usually unleashed by external “porphyrinogenic” triggers, which are thought to cause an increased metabolic demand for heme. During APAs, the heme precursors δ-aminolevulinic acid (ALA) and porphobilinogen (PBG) accumulate in the bloodstream and urine. Even though several hypotheses have been developed to explain the protean clinical picture of APAs, the exact mechanism of neuronal damage in AHPs is still a matter of debate. In recent decades, a role has been proposed for oxidative damage caused by ALA, mitochondrial and synaptic ALA toxicity, dysfunction induced by relative heme deficiency on cytochromes and other hemeproteins (i.e., nitric oxide synthases), pyridoxal phosphate functional deficiency, derangements in the metabolic pathways of tryptophan, and other factors. Since the pathway leading to the biosynthesis of heme is inscribed into a complex network of interactions, which also includes some fundamental processes of basal metabolism, a disruption in any of the steps of this pathway is likely to have multiple pathogenic effects. Here, we aim to provide a comprehensive review of the current evidence regarding the mechanisms of neuronal damage in AHPs.

2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Elvira Ferrés-Amat ◽  
Jordi Prats-Armengol ◽  
Isabel Maura-Solivellas ◽  
Eduard Ferrés-Amat ◽  
Javier Mareque-Bueno ◽  
...  

Intraosseous arteriovenous malformations (AVMs) in the head and neck region are uncommon. There are several types and they can have a wide range of clinical presentations. Depending on the blood flow through the AVM, the treatment may be challenging for the attending team and may lead to life-threatening hemorrhages. A clinical case report is presented. A 9-year-old girl, seen for gingival bleeding during oral hygiene, was found to have a high-flow AVM located within and around the mandible. Two-stage treatment consisted of intra-arterial embolization followed by intraoral injection of a sclerosing agent 8 weeks later. At the 8-year follow-up, imaging study showed no evidence of recurrent lesion inside or outside the bone. The final outcome is a correct occlusion with a symmetric facial result. This case shows that conservative treatment may be the first treatment option mostly in children. Arteriography and transcortical injection were enough to control the AVM.


1997 ◽  
Vol 3 (5) ◽  
pp. 314-327 ◽  
Author(s):  
Raymond T. Bartus

Calpain's general function and pathogenic role in the CNS are reviewed. Collectively, the literature indicates that calpain proteolysis plays a common and important role in a variety of acute neurodegenerative conditions, including focal ischemia (stroke), global ischemia, traumatic brain injury, and spinal cord injury. This evidence indicates that 1) calpain is activated in an abnormally sustained fashion during cellular events commonly associated with neurodegeneration (e.g., excessive interstitial glutamate and cytosolic calcium); 2) many of calpain's preferred substrates are degraded as important components in these neurodegenerative conditions; 3) calpain activation occurs early in the pathogenic cascade of each, prior to onset of substantial cell death; and 4) calpain inhibitors can effectively reduce the severity of neuronal damage and loss of function normally associated with these acute neurodegenerative perturbations. Calpain proteolysis is also implicated in chronic neurodegenerative diseases, with the strength of current evidence varying among specific diseases. The evidence accumulated for a plausible role in Alzheimer's disease (AD) is currently the strongest. For example, empirical links have been established between abnormal calpain proteolysis and 1) the cellular formation of classic Alzheimer's pathology, such as β-amyloid plaques, neurofibrillary tangles, and Alz-50 immunoreactivity; 2) the brain regions with greatest concentrations of AD-related pathology; and 3) the degeneration of key brain pathways vulnerable in the early stages of the disease. Similar, though less extensive, evidence exists for a potential role of abnormal calpain proteolysis in Parkinson's disease. Finally, for several other chronic neurodegenerative conditions (e.g., Huntington's disease and amyotrophic lateral sclerosis), early evidence is emerging that calpain may also play some pathogenic role. Thus, these data support the possibility that uncontrolled calpain proteolysis may contribute to and/or accelerate the loss of neurons associated with a wide range of neurodegenerative conditions and may, therefore, represent an important, final common cytotoxic pathway for many diverse forms of neurodegeneration. NEUROSCIENTIST 3:314–327, 1997


2020 ◽  
Vol 12 (Suppl. 1) ◽  
pp. 63-69
Author(s):  
Kar Foo Lau ◽  
Tsun-Haw Toh ◽  
Khairul Azmi Abdul Kadir ◽  
Mei-Ling Sharon Tai  ◽  
Kay Sin Tan

Cerebral venous thrombosis (CVT) is a rare cause of stroke worldwide with a wide range of clinical presentations. Anticoagulation therapy has been regarded as the first line of management of CVT to prevent the progression of thrombosis and to re-establish the venous flow. We present a case of severe CVT who did not respond to conventional anticoagulation therapy but responded well to mechanical thrombectomy (MT). This report highlights the features of CVT to consider for early MT.


2020 ◽  
Vol 21 (2) ◽  
pp. 97-109 ◽  
Author(s):  
Ana P. dos Santos ◽  
Tamara G. de Araújo ◽  
Gandhi Rádis-Baptista

Venom-derived peptides display diverse biological and pharmacological activities, making them useful in drug discovery platforms and for a wide range of applications in medicine and pharmaceutical biotechnology. Due to their target specificities, venom peptides have the potential to be developed into biopharmaceuticals to treat various health conditions such as diabetes mellitus, hypertension, and chronic pain. Despite the high potential for drug development, several limitations preclude the direct use of peptides as therapeutics and hamper the process of converting venom peptides into pharmaceuticals. These limitations include, for instance, chemical instability, poor oral absorption, short halflife, and off-target cytotoxicity. One strategy to overcome these disadvantages relies on the formulation of bioactive peptides with nanocarriers. A range of biocompatible materials are now available that can serve as nanocarriers and can improve the bioavailability of therapeutic and venom-derived peptides for clinical and diagnostic application. Examples of isolated venom peptides and crude animal venoms that have been encapsulated and formulated with different types of nanomaterials with promising results are increasingly reported. Based on the current data, a wealth of information can be collected regarding the utilization of nanocarriers to encapsulate venom peptides and render them bioavailable for pharmaceutical use. Overall, nanomaterials arise as essential components in the preparation of biopharmaceuticals that are based on biological and pharmacological active venom-derived peptides.


2020 ◽  
Vol 12 ◽  
Author(s):  
Nihar Ranjan Biswal

Background: Surfactant adsorption at the interfaces (solid–liquid, liquid–air, or liquid–liquid) is receiving considerable attention from a long time due to its wide range of practical applications. Objective: Specifically wettability of solid surface by liquids is mainly measured by contact angle and has many practical importances where solid–liquid systems are used. Adsorption of surfactants plays an important role in the wetting process. The wetting behaviours of three plant-based natural surfactants (Reetha, Shikakai, and Acacia) on the glass surface are compared with one widely used nonionic synthetic surfactant (Triton X-100) and reported in this study. Methods: The dynamic contact angle study of three different types of plant surfactants (Reetha, Shikakai and Acacia) and one synthetic surfactant (Triton X 100) on the glass surface has been carried out. The effect of two different types of alcohols such as Methanol and amyl alcohol on wettability of shikakai, as it shows little higher value of contact angle on glass surface has been measured. Results: The contact angle measurements show that there is an increase in contact angle from 47° (pure water) to 67.72°, 65.57°, 68.84°, and 68.79° for Reetha, Acacia, Shikakai, and Triton X-100 respectively with the increase in surfactant concentration and remain constant at CMC. The change in contact angle of Shikakai-Amyl alcohol mixtures are slightly different than that of methanol-Shikakai mixture, mostly there is a gradual increase in contact angle with the increasing in alcohol concentration. Conclusion: There is no linear relationship between cos θ and inverse of surface tension. There was a linear increase in surface free energy results with increase in concentration as more surfactant molecules were adsorbing at the interface enhancing an increase in contact angle.


Author(s):  
_______ Archana ◽  
Charu Datta ◽  
Pratibha Tiwari

Degradation of environment is one of the most serious challenges before the mankind in today’s world. Mankind has been facing a wide range of problem arising out of the degradation of environment. Not only the areas under human inhabitation, but the areas of the planet without human population have also been suffering from these problems. As the population increase day by day, the amenities are not improved simultaneously. With the advancement of science and technologies the needs of human beings has been changing rapidly. As a result different types of environmental problems have been rising. Environmental degradation is a wide- reaching problem and it is likely to influence the health of human population is great. It may be defined the deterioration of the environment through depletion of resources such as air, water, and soil. The destruction of ecosystem and extinction of wildlife. Environmental degradation has occurred due to the recent activities in the field of socio-economic, institute and technology. Poverty still remains a problem as the root of several environmental problems to create awareness among the people about the ill effect of environmental pollution. In the whole research it is clear that all factors of environmental degradation may be reduced through- Framing the new laws on environmental degradation, Environment friend policy, Controlling all the ways and means of noise, air, soil and water pollution, Through growing more and more trees and by adapting the proper sanitation policy.  


2020 ◽  
Vol 21 (15) ◽  
pp. 5475 ◽  
Author(s):  
Manuela Pennisi ◽  
Giuseppe Lanza ◽  
Luca Falzone ◽  
Francesco Fisicaro ◽  
Raffaele Ferri ◽  
...  

Increasing evidence suggests that Severe Acute Respiratory Syndrome-coronavirus-2 (SARS-CoV-2) can also invade the central nervous system (CNS). However, findings available on its neurological manifestations and their pathogenic mechanisms have not yet been systematically addressed. A literature search on neurological complications reported in patients with COVID-19 until June 2020 produced a total of 23 studies. Overall, these papers report that patients may exhibit a wide range of neurological manifestations, including encephalopathy, encephalitis, seizures, cerebrovascular events, acute polyneuropathy, headache, hypogeusia, and hyposmia, as well as some non-specific symptoms. Whether these features can be an indirect and unspecific consequence of the pulmonary disease or a generalized inflammatory state on the CNS remains to be determined; also, they may rather reflect direct SARS-CoV-2-related neuronal damage. Hematogenous versus transsynaptic propagation, the role of the angiotensin II converting enzyme receptor-2, the spread across the blood-brain barrier, the impact of the hyperimmune response (the so-called “cytokine storm”), and the possibility of virus persistence within some CNS resident cells are still debated. The different levels and severity of neurotropism and neurovirulence in patients with COVID-19 might be explained by a combination of viral and host factors and by their interaction.


2021 ◽  
Vol 11 (7) ◽  
pp. 3209
Author(s):  
Karla R. Borba ◽  
Didem P. Aykas ◽  
Maria I. Milani ◽  
Luiz A. Colnago ◽  
Marcos D. Ferreira ◽  
...  

Portable spectrometers are promising tools that can be an alternative way, for various purposes, of analyzing food quality, such as monitoring in a few seconds the internal quality during fruit ripening in the field. A portable/handheld (palm-sized) near-infrared (NIR) spectrometer (Neospectra, Si-ware) with spectral range of 1295–2611 nm, equipped with a micro-electro-mechanical system (MEMs), was used to develop prediction models to evaluate tomato quality attributes non-destructively. Soluble solid content (SSC), fructose, glucose, titratable acidity (TA), ascorbic, and citric acid contents of different types of fresh tomatoes were analyzed with standard methods, and those values were correlated to spectral data by partial least squares regression (PLSR). Fresh tomato samples were obtained in 2018 and 2019 crops in commercial production, and four fruit types were evaluated: Roma, round, grape, and cherry tomatoes. The large variation in tomato types and having the fruits from distinct years resulted in a wide range in quality parameters enabling robust PLSR models. Results showed accurate prediction and good correlation (Rpred) for SSC = 0.87, glucose = 0.83, fructose = 0.87, ascorbic acid = 0.81, and citric acid = 0.86. Our results support the assertion that a handheld NIR spectrometer has a high potential to simultaneously determine several quality attributes of different types of tomatoes in a practical and fast way.


Polymers ◽  
2021 ◽  
Vol 13 (10) ◽  
pp. 1598
Author(s):  
Chih-Yu Chung ◽  
Yu-Ju Chen ◽  
Chia-Hui Kang ◽  
Hung-Yun Lin ◽  
Chih-Ching Huang ◽  
...  

Carbon quantum dots (CQDs) are emerging novel nanomaterials with a wide range of applications and high biocompatibility. However, there is a lack of in-depth research on whether CQDs can cause acute or long-term adverse reactions in aquatic organisms. In this study, two different types of CQDs prepared by ammonia citrate and spermidine, namely CQDAC and CQDSpd, were used to evaluate their biocompatibilities. In the fish embryo acute toxicity test (FET), the LD50 of CQDAC and CQDSpd was about 500 and 100 ppm. During the stage of eleutheroembryo, the LD50 decreased to 340 and 55 ppm, respectively. However, both CQDs were quickly eliminated from embryo and eleutheroembryo, indicating a lack of bioaccumulation. Long-term accumulation of CQDs was also performed in this study, and adult zebrafish showed no adverse effects in 12 weeks. In addition, there was no difference in the hatchability and deformity rates of offspring produced by adult zebrafish, regardless of whether they were fed CQDs or not. The results showed that both CQDAC and CQDSpd have low toxicity and bioaccumulation to zebrafish. Moreover, the toxicity assay developed in this study provides a comprehensive platform to assess the impacts of CQDs on aquatic organisms in the future.


Materials ◽  
2021 ◽  
Vol 14 (3) ◽  
pp. 513
Author(s):  
Anna Rabajczyk ◽  
Maria Zielecka ◽  
Krzysztof Cygańczuk ◽  
Łukasz Pastuszka ◽  
Leszek Jurecki

A recent trend in the field of membrane research is the incorporation of nanoparticles into polymeric membranes, which could produce synergistic effects when using different types of materials. This paper discusses the effect of the introduction of different nanometals such as silver, iron, silica, aluminum, titanium, zinc, and copper and their oxides on the permeability, selectivity, hydrophilicity, conductivity, mechanical strength, thermal stability, and antiviral and antibacterial properties of polymeric membranes. The effects of nanoparticle physicochemical properties, type, size, and concentration on a membrane’s intrinsic properties such as pore morphology, porosity, pore size, hydrophilicity/hydrophobicity, membrane surface charge, and roughness are discussed, and the performance of nanocomposite membranes in terms of flux permeation, contaminant rejection, and antifouling capability are reviewed. The wide range of nanocomposite membrane applications including desalination and removal of various contaminants in water-treatment processes are discussed.


Sign in / Sign up

Export Citation Format

Share Document