Your browser doesn't support javascript.
loading
Show: 20 | 50 | 100
Results 1 - 20 de 19.859
Filter
Add more filters








Publication year range
1.
Angew Chem Int Ed Engl ; : e202409256, 2024 Aug 01.
Article in English | MEDLINE | ID: mdl-39088255

ABSTRACT

Developing an anode material that has better performance efficiency than commercial graphite while keeping the features of economic scalability and environmental safety is highly desirable yet challenging. MOFs are a promising addition to the ongoing efforts, however, the relatively poor performance, chemical instability, and large-scale economic production of efficiency-proven pristine MOFs restrict their utility in real-life energy storage applications. Furthermore, hierarchical porosity for lucid mass diffusion, high-density lithiophilic sites are some of the structural parameters for improving the electrode performance. Herein, we have demonstrated the potential of economically scalable salicylaldehydate 3D-conjugated-MOF (Fe-Tp) as a high-performance anode in Li-ion batteries: the anode-specific capacity achieved up to 1447 mA h g-1 at 0.1 A g-1 and 89% of cyclic stability after 500 cycles at 1.0 A g-1.for pristine MOF. More importantly, incorporating 10% Fe-Tp doping in commercial graphite (MOFite) significantly enhanced lithium storage, doubling capacitance after 400 cycles. It signifies the potential practical utility of Fe-Tp as a performance booster for commercial anode material.

2.
Prev Med Rep ; 44: 102813, 2024 Aug.
Article in English | MEDLINE | ID: mdl-39099773

ABSTRACT

Objectives: To examine factors that differentiate firearm owners who endorse specific reasons for secure and unsecure firearm storage. Methods: A subsample of firearm-owning adults (n = 3,119) drawn from a representative sample of adults (n = 7,785) residing in nine US states participated in an online survey. Results: The most common reason for not always using a gun safe was concerns that they render firearms too slow to access during an emergency (60.2%). The most common reasons for current firearm storage were ensuring ready access in case of emergency (59.7%) and preventing access by children and adolescents (44.6%). Firearm owners varied on their reasons based upon current storage habits, reasons for ownership, and the presence of firearms in the home. Conclusions: Firearm owners with children in the home are more likely to select storage methods they feel will prevent access by vulnerable individuals, whereas those who own for defensive purposes and those who store firearms loaded and unlocked are more likely to endorse storing their firearms for quick home defense and convenience and to see gun safes as unnecessary obstacles.

3.
Adv Clin Chem ; 122: 1-52, 2024.
Article in English | MEDLINE | ID: mdl-39111960

ABSTRACT

Glycosaminoglycans (GAGs) are sulfated polysaccharides comprising repeating disaccharides, uronic acid (or galactose) and hexosamines, including chondroitin sulfate, dermatan sulfate, heparan sulfate, and keratan sulfate. Hyaluronan is an exception in the GAG family because it is a non-sulfated polysaccharide. Lysosomal enzymes are crucial for the stepwise degradation of GAGs to provide a normal function of tissues and extracellular matrix (ECM). The deficiency of one or more lysosomal enzyme(s) results in the accumulation of undegraded GAGs, causing cell, tissue, and organ dysfunction. Accumulation of GAGs in various tissues and ECM results in secretion into the circulation and then excretion in urine. GAGs are biomarkers of certain metabolic disorders, such as mucopolysaccharidoses (MPS) and mucolipidoses. GAGs are also elevated in patients with various conditions such as respiratory and renal disorders, fatty acid metabolism disorders, viral infections, vomiting disorders, liver disorders, epilepsy, hypoglycemia, myopathy, developmental disorders, hyperCKemia, heart disease, acidosis, and encephalopathy. MPS are a group of inherited metabolic diseases caused by the deficiency of enzymes required to degrade GAGs in the lysosome. Eight types of MPS are categorized based on lack or defect in one of twelve specific lysosomal enzymes and are described as MPS I through MPS X (excluding MPS V and VIII). Clinical features vary with the type of MPS and clinical severity of the disease. This chapter addresses the historical overview, synthesis, degradation, distribution, biological role, and method for measurement of GAGs.


Subject(s)
Glycosaminoglycans , Mucopolysaccharidoses , Humans , Mucopolysaccharidoses/metabolism , Glycosaminoglycans/metabolism , Animals
4.
Dent Mater ; 2024 Aug 07.
Article in English | MEDLINE | ID: mdl-39112293

ABSTRACT

OBJECTIVE: Acyclovir (ACY) is used to treat oral viral herpes but has low solubility and bioavailability. Stearic acid (SA) is lipophilic and can be combined with drugs. Therefore, this study aimed to characterize the properties of SA nanoparticles in increasing the cellular uptake of ACY by oral epithelial cells. The hypothesis was that SA nanoparticles increase sustained ACY release, are stable, and increase drug uptake. METHODS: The production parameters (duration and amplitude of sonication) were optimized to produce solid lipid nanoparticles (SLN) of SA-containing ACY. Particle stability was characterized under different storage conditions (4 °C and 37 °C for 1, 15, and 45 days). SLN were further characterized for their pharmacokinetic profile, cytotoxicity, in vitro permeability, and ability to modulate gene expression and promote ACY uptake by oral epithelial cells. RESULTS: Pharmacokinetic studies revealed sustained and diffusional release of ACY from the SLN, with an initial burst release of 15 min. After 45 d of storage, SLN kept at both 4 °C and 37 °C showed a maximum release of > 90 % of the drug at 120 min. Cells treated with SLN presented a significantly higher intracellular drug content than those treated with ACY and significantly increased the genetic expression of TJP-1, OCLN, and ECAD. SIGNIFICANCE: The hypothesis was accepted as SA nanoparticles containing ACY can sustain drug delivery and enhance its absorption into epithelial cells. Therefore, SA nanoparticles are promising for improving ACY uptake in treating oral herpes and other infections caused by HSV-1.

5.
Article in English | MEDLINE | ID: mdl-39112900

ABSTRACT

This study aims to use beeswax, a readily available and cost-effective organic material, as a novel phase change material (PCM) within blends of low-density polyethylene (LDPE) and styrene-b-(ethylene-co-butylene)-b-styrene (SEBS). LDPE and SEBS act as support materials to prevent beeswax leakage. The physicochemical properties of new blended phase change materials (B-PCM) were determined using an X-ray diffractometer and an infrared spectrometer, confirming the absence of a chemical reaction within the materials. A scanning electron microscope was used for microstructural analysis, indicating that the interconnection of the structure allowed better thermal conductivity. Thermal gravimetric analysis revealed enhanced thermal stability for the B-PCM when combined with SEBS, especially within its operating temperature range. Analysis of phase change temperature and latent heat with differential scanning calorimetry showed no major difference in the melting point of the various PCM blends created. During the melting/solidification process, the B-PCMs possess excellent performance as characterized by W70/P30 (112.45 J.g-1) > W70/P20/S10 (94.28 J.g-1) > W70/P10/S20 (96.21 J.g-1) of latent heat storage. Additionally, the blends tend to reduce supercooling compared to pure beeswax. During heating and cooling cycles, the B-PCM exhibited minimal leakage and degradation, especially in blends containing SEBS. In comparison to the rapid temperature drop observed during the cooling process of W70/P30, the temperature decline of W70/P30 was slower and longer, as demonstrated by infrared thermography. The addition of LDPE to the PCM reduced melting time, indicating an improvement in the thermal energy storage reaction time to the demand. According to the obtained findings, increasing the SEBS concentration in the composite increased the thermal stability of the resulting PCM blends significantly. Despite the challenges mentioned earlier, SEBS proved to be an effective encapsulating material for beeswax, whereas LDPE served well as a supporting material. Leak tests were performed to find the ideal mass ratio, and weight loss was analyzed after multiple cycles of cooling and heating at 70 °C. The morphology, thermal characteristics, and chemical composition of the beeswax/LDPE/SEBS composite were all examined. Beeswax proves to be a highly effective phase change material for storing thermal energy within LDPE/SEBS blends.

6.
BJU Int ; 2024 Aug 07.
Article in English | MEDLINE | ID: mdl-39113489

ABSTRACT

OBJECTIVE: To test for differences in recovery of lower urinary tract symptoms (LUTS) between patients with storage-positive vs -negative symptoms after laser enucleation of the prostate (LEP). PATIENTS AND METHODS: Consecutive storage-positive (severe storage symptoms, International Prostate Symptom Score [IPSS] storage subscore >8) vs storage-negative patients treated with LEP (November 2017-September 2022) within our tertiary-care database were identified. Mixed linear models tested for changes in IPSS and quality of life (QoL) at 1, 3 and 12 months after LEP. Multiple linear regression models tested for LUTS and QoL recovery risk factors at 1, 3 and 12 months. RESULTS: Of 291 study patients, 180 (62%) had storage-positive symptoms. There were no differences between storage-positive and -negative patients in mean adjusted total IPSS, IPSS-storage, IPSS-voiding and QoL at 12 months after LEP. In multiple linear regression models, storage-positive status was identified as a risk factor for higher IPSS at 1 month (ß coefficient 2.98, P = 0.004) and 3 months (ß coefficient 2.24, P = 0.04), as well as for more unfavourable QoL at 1 month (ß coefficient 0.74, P = 0.006) and 3 months (ß coefficient 0.73, P = 0.004) after LEP. Conversely, at 12 months there were no differences between storage-positive vs -negative patients. CONCLUSION: Storage-positive patients appear to experience similar long-term benefits from LEP compared to storage-negative patients. However, significant storage symptoms are associated with higher total IPSS and less favourable QoL at 1 and 3 months after LEP. These findings advocate for the consideration of LEP also in storage-positive cases with the need for thorough patient education especially in the initial post-LEP period.

7.
Heliyon ; 10(14): e33944, 2024 Jul 30.
Article in English | MEDLINE | ID: mdl-39114005

ABSTRACT

It is challenging to accurately model the overall uncertainty of the power system when it is connected to large-scale intermittent generation sources such as wind and photovoltaic generation due to the inherent volatility, uncertainty, and indivisibility of renewable energy. Deep reinforcement learning (DRL) algorithms are introduced as a solution to avoid modeling the complex uncertainties and to adapt the fluctuation of uncertainty by interacting with the environment and using feedback to continuously improve their strategies. However, the large-scale nature and uncertainty of the system lead to the sparse reward problem and high-dimensional space issue in DRL. A hierarchical deep reinforcement learning (HDRL) scheme is designed to decompose the process of solving this problem into two stages, using the reinforcement learning (RL) agent in the global stage and the heuristic algorithm in the local stage to find optimal dispatching decisions for power systems under uncertainty. Simulation studies have shown that the proposed HDRL scheme is efficient in solving power system economic dispatch problems under both deterministic and uncertain scenarios thanks to its adaptation system uncertainty, and coping with the volatility of uncertain factors while significantly improving the speed of online decision-making.

8.
J Adv Vet Anim Res ; 11(2): 317-322, 2024 Jun.
Article in English | MEDLINE | ID: mdl-39101074

ABSTRACT

Objective: This study investigated the application of Jicama starch (Pachyrhizus erosus L.) as a stabilizing agent to enhance the longevity and integrity of fermented milk. Materials and Methods: Lactobacillus plantarum SN13T (6 gm/100 ml) and Jicama starch (2 gm/100 ml) were added into pasteurized milk (65°C, 30 min) and then incubated under anaerobic conditions at 37°C for 18 h. The fermented milk was stored at 4°C. The evaluation on proximate composition, pH, titratable acidity (TA), viscosity, water holding capacity (WHC), syneresis, total lactic acid bacteria (LAB), and hedonic sensory evaluation was conducted at 1, 7, 14, 21, and 28 days of storage. Results: Throughout the storage period, fermented milk enriched with Jicama starch significantly (p < 0.05) increased pH, TA, population dynamics of LAB, viscosity, WHC, and syneresis. It effectively sustained WHC and mitigated syneresis, thus ensuring the preservation of vital product quality. Furthermore, the quantity of LAB within the fermented milk consistently met the probiotic threshold of 84.50 × 108 CFU/ml. The hedonic sensory evaluation results indicated that fermented milk showed consistent sensory attributes throughout storage, except for overall acceptance, which declined on day 28. Conclusion: The addition of Jicama starch revealed a promising health probiotic product, presenting a viable avenue for delivering probiotic benefits to consumers while maintaining the palatability and efficacy of the product.

9.
Small ; : e2404893, 2024 Aug 06.
Article in English | MEDLINE | ID: mdl-39105465

ABSTRACT

Vanadium-based compounds have attracted significant attention as cathodes for aqueous zinc metal batteries (AZMBs) because of their remarkable advantages in specific capacities. However, their low diffusion coefficient for zinc ions and structural collapse problems lead to poor rate capability and cycle stability. In this work, bilayered Sr0.25V2O5·0.8H2O (SVOH) nanowires are first reported as a highly stable cathode material for rechargeable AZMBs. The synergistic pillaring effect of strontium ions and water molecules improves the structural stability and ion transport dynamics of vanadium-based compounds. Consequently, the SVOH cathode exhibits a high capacity of 325.6 mAh g-1 at 50 mA g-1, with a capacity retention rate of 72.6% relative to the maximum specific capacity at 3.0 A g-1 after 3000 cycles. Significantly, a unique single-nanowire device is utilized to demonstrate the excellent conductivity of the SVOH cathode directly. Additionally, the energy storage mechanism of zinc insertion and extraction is investigated using a variety of advanced in situ and ex situ analysis techniques. This method of ion intercalation to improve electrochemical performance will further promote the development of AZMBs in large-scale applications.

10.
Environ Sci Technol ; 2024 Aug 06.
Article in English | MEDLINE | ID: mdl-39105763

ABSTRACT

We present methods and insights for the design of CO2 capture, transport, and storage systems for industrial facilities with a case study focus on Louisiana. Our analytical framework includes (1) evaluating the scale and concentration of capturable CO2 emissions at individual facilities for the purpose of estimating the cost of CO2 capture retrofits that utilize various energy supply sources to meet parasitic demands; (2) screening to identify potential CO2 storage sites and estimate their capacities, injectivities, and costs; and (3) designing cost-minimized trucking or pipeline infrastructure connecting CO2 capture plants with storage sites, considering existing land uses, demographics, and a variety of social and environmental justice factors. Estimated levelized costs of capture at Louisiana's 190 industrial facilities range from below $50/tCO2 to above $500/tCO2, depending on facility-specific features. We identified 98 potential storage sites with storage costs ranging from $8 to $17/tCO2. We find that in most situations, pipelines are the least-costly mode of CO2 transport. When industrial facilities in a region share pipelines, aggregate pipeline mileage and average transport costs are dramatically lower than without sharing. Shared pipeline networks designed to avoid disadvantaged communities require right-of-way areas compared to those for networks that transect such communities, but result in 25% higher average per-tonne transport cost.

11.
Inn Med (Heidelb) ; 2024 Aug 06.
Article in German | MEDLINE | ID: mdl-39105759

ABSTRACT

Fabry's disease is a rare X chromosome-linked inherited lysosomal storage disease characterized by insufficient metabolism of the substrate globotriaosylceramide (Gb3) due to reduced alpha-galactosidase A (AGAL) activity. Lysosomal Gb3 accumulation causes a multisystemic disease which, if untreated, reduces the life expectancy in females and males by around 10 and 20 years, respectively, due to progressive renal dysfunction, hypertrophic cardiomyopathy, cardiac arrhythmia and early occurrence of cerebral infarction. The diagnosis is confirmed by determining the reduced AGAL activity in leukocytes in males and molecular genetic detection of a -mutation causing the disease in females. The treatment comprises enzyme replacement therapy (ERT), agalsidase alfa, 0.2 mg/kg body weight (BW), agalsidase beta 1.0 mg/kg BW or pegunigalsidase alfa 1.0 mg/kg BW every 2 weeks i.v. or oral chaperone therapy (one capsule of migalastat 123 mg every other day) in the presence of amenable mutations. This article summarizes the data on the treatment of Fabry's disease and on complications in practice. The current guideline recommendations are addressed and new study results that could expand the therapeutic repertoire in the future are discussed.

12.
ACS Nano ; 2024 Aug 06.
Article in English | MEDLINE | ID: mdl-39105765

ABSTRACT

Two-dimensional (2D) materials with excellent properties and widespread applications have been explosively investigated. However, their conventional synthetic methods exhibit concerns of limited scalability, complex purification process, and incompetence of prohibiting their restacking. The blowing strategy, characterized by gas-template, low-cost, and high-efficiency, presents a valuable avenue for the synthesis of 2D-based foam materials and thereby addresses these constraints. Whereas, its comprehensive introduction has been rarely outlined so far. This review commences with a synopsis of the blowing strategy, elucidating its development history, the statics and kinetics of the blowing process, and the choice of precursor and foaming agents. Thereafter, we dwell at length on across-the-board foams enabled by the blowing route, like BxCyNz foams, carbon foams, and diverse composite foams consisting of carbon and metal compounds. Following that, a wide-ranging evaluation of the functionality of the foam products in fields such as energy storage, electrocatalysis, adsorption, etc. is discussed, revealing their distinctive strength originated from the foam structure. Finally, after concluding the current progress, we provide some personal discussions on the existing challenges and future research priorities in this rapidly developing method.

13.
Article in English | MEDLINE | ID: mdl-39106014

ABSTRACT

The incorporation of renewable energy resources (RERs) into smart city through hybrid microgrid (HMG) offers a sustainable solution for clean energy. The HMG architecture also involves linking the AC-microgrid and DC-microgrid through bidirectional interconnection converters (ICC). This HMG combines AC sources like wind-DFIG with DC sources such as solar PV and solid oxide fuel cell (SOFC), supported by battery energy storage systems (BESS) and hydrogen storage units (HSU). The HSU can generate and store hydrogen during RER surplus. This stored hydrogen can be further employed for production of electrical power along with numerous other applications. The HSU is emerged as a competent tool which can be utilised alone/in combination with BESS to enhance the system reliability. Harvesting power from clean and green sources requires its optimal operation and control while feeding to the existing grid. The existing strategies of controlling ICC are complex and not efficient; hence, a novel intelligent scaled droop control structure (SDCS) is proposed, utilizing frequency, DC voltage, and active power. The SDCS regulate voltage and frequency in both islanded mode (IM) and grid connected mode (GCM) of HMG. Experimental validation demonstrates its simplicity and effectiveness, making it suitable for smart city environments, ensuring uninterrupted power for critical loads with improved air quality.

14.
Sci Rep ; 14(1): 18115, 2024 Aug 05.
Article in English | MEDLINE | ID: mdl-39103460

ABSTRACT

The mechanism of Y on H/H2 adsorption performance of Mg17Al12 were studied by the density functional theory. We obtained that for the Y-adsorbed systems, Y tended to occupy on the bridge site between adjacent Mg atoms. For the Y-substituted surfaces, Y atoms inclined to replace Mg atoms on the surfaces. We found that hydrogen (H/H2) absorption on the Mg17Al12(110) systems were improved by adding Y, the order of adsorption energy was as follows: clean Mg17Al12(110) > the Y-substituted surfaces > the Y-adsorbed surfaces. In addition, H2 molecules could dissociate on the Y-containing systems without barrier energy. Electronic properties showed that for H2 adsorption, the s states of atomic H mainly hybridized with the d states of Y. The formations of the Y-H bonds and the interactions between Y and H atoms could expound the mechanism for the promoted hydrogenation performance of the Y-containing surfaces.

15.
iScience ; 27(8): 110433, 2024 Aug 16.
Article in English | MEDLINE | ID: mdl-39108739

ABSTRACT

Lignin is an unlimited resource of an aromatic natural polymer useful for multiple applications, though its use remains limited due to the lack of technology developments. The introduction of lignin as coating of fibers and textile for composites is being recently studied with very promising results. Here, commercial woven carbon fibers (WCFs) have been superficially modified following an easy approach to introduce lignin and carbonize it to enhance the electrochemical performance of the fibers. The modified carbon fibers containing lignin were tested as electrodes using KCl 3 M as electrolyte, reaching 5 F/g at 10 mVs-1. A symmetric supercapacitor was fabricated using these electrodes, and a proof of concept based on a red and yellow light-emitting diode (LED) powered on and a bending test were done. The supercapacitor was able to recover 99% of its capacitance when returned to its original unbent position.

16.
Food Chem ; 460(Pt 3): 140680, 2024 Jul 31.
Article in English | MEDLINE | ID: mdl-39106756

ABSTRACT

Hard to cook is a textural defect that affects the nutritional quality of beans stored under adverse temperature and humidity conditions. This defect is related to intrinsic characteristics such as seed coat thickness, composition and microstructure. The aim of the present study was to evaluate the chemical and microstructural characteristics of common bean (Phaseolus vulgaris L.) during 270 days of post-harvest storage at 30 °C and 70% relative humidity. Microstructural analysis revealed alteration of the cotyledon cell wall and seed coat affecting seed viability and restricting seedling emergence. The seed coat thickness contraction from 105.79 µm to 97.35 µm (270 days). Changes are related with the protein bodies migration from cotyledons to seed coat. An increase in neutral detergent fiber and the presence of CaOx crystals were observed, which confer rigidity to the seed coat and affect water diffusion after 150 days causing permeability changes that contributed to seed hardening.

18.
Adv Sci (Weinh) ; : e2307583, 2024 Aug 06.
Article in English | MEDLINE | ID: mdl-39107963

ABSTRACT

Water molecules confined in nanoscale spaces of 2D graphene layers have fascinated researchers worldwide for the past several years, especially in the context of energy storage applications. The water molecules exchanged along with ions during the electrochemical process can aid in wetting and stabilizing the layered materials resulting in an anomalous enhancement in the performance of supercapacitor electrodes. Engineering of 2D carbon electrode materials with various functionalities (oxygen (─O), fluorine (─F), nitrile (─C≡N), carboxylic (─COOH), carbonyl (─C═O), nitrogen (─N)) can alter the ion/water organization in graphene derivatives, and eventually their inherent ion storage ability. Thus, in the current study, a comparative set of functionalized graphene derivatives-fluorine-doped cyanographene (G-F-CN), cyanographene (G-CN), graphene acid (G-COOH), oxidized graphene acid (G-COOH (O)) and nitrogen superdoped graphene (G-N) is systematically evaluated toward charge storage in various aqueous-based electrolyte systems. Differences in functionalization on graphene derivatives influence the electrochemical properties, and the real-time mass exchange during the electrochemical process is monitored by electrochemical quartz crystal microbalance (EQCM). Electrogravimetric assessment revealed that oxidized 2D acid derivatives (G-COOH (O)) are shown to exhibit high ion storage performance along with maximum water transfer during the electrochemical process. The complex understanding of the processes gained during supercapacitor electrode charging in aqueous electrolytes paves the way toward the rational utilization of graphene derivatives in forefront energy storage applications.

19.
Macromol Rapid Commun ; : e2400482, 2024 Aug 06.
Article in English | MEDLINE | ID: mdl-39108056

ABSTRACT

Digitally-encoded poly(phosphodiesters) (d-PPDE) with highly complex primary structures are evaluated for layer-by-layer (LbL) assembly. To be easily decoded by mass spectrometry (MS), these digital polymers contain many different monomers: 2 coding units allowing binary encryption, 1 cleavable spacer allowing controlled MS fragmentation, and 3 mass tags allowing fragment identification. These complex heteropolymers are therefore composed of 6 different motifs. Despite this strong sequence heterogeneity, it is found that they enable a highly controlled LbL film formation. For instance, a regular growth is observed when alternating the deposition of negatively-charged d-PPDE and positively-charged poly(allyl amine hydrochloride) (PAH). Yet, in this approach, the interdistance between consecutive coded d-PPDE layers remains relatively small, which may be an issue for data storage applications, especially for the selective decoding of the stored information. Using poly(sodium 4-styrene sulfonate) (PSS) as an intermediate non-coded polyanion, it is shown that a controlled interdistance between d-PPDE layers can be easily achieved, while still maintaining a regular LbL growth. Last but not least, it is found in this work that d-PPDE of relatively small molecular weight (i.e., significantly smaller than those of PAH and PSS) still enables a controlled LbL assembly.

20.
Mol Ther ; 2024 Aug 05.
Article in English | MEDLINE | ID: mdl-39108096

ABSTRACT

Farber disease (FD) and spinal muscular atrophy with progressive myoclonic epilepsy (SMA-PME) are ultra-rare lysosomal storage disorders caused by deficient acid ceramidase (ACDase) activity. Although both conditions are caused by mutations in the ASAH1 gene, clinical presentations differ considerably. FD patients usually die in childhood while SMA-PME patients can live until adulthood. There is no treatment for FD or SMA-PME. Hematopoietic stem cell transplantation (HSCT) and gene therapy strategies for the treatment of ACDase deficiency are being investigated. We have previously generated and characterized mouse models of both FD and SMA-PME that recapitulate the symptoms described in patients. Here, we show that HSCT improves lifespan, behavior, hematopoietic system anomalies, plasma cytokine levels, and significantly reduces histiocytic infiltration and ceramide accumulation throughout the tissues investigated, including the CNS, in both models of ACDase deficient mice. HSCT was also successful in preventing lesion development and significant demyelination of the spinal cord seen in SMA-PME mice. Importantly, we note that only early and generally pre-symptomatic treatment was effective and kidney impairment was not improved in either model.

SELECTION OF CITATIONS
SEARCH DETAIL