Abstract：
:Ataxia is a common clinical feature in inherited metabolic disorders. There are more than 150 inherited metabolic disorders in patients presenting with ataxia in addition to global developmental delay, encephalopathy episodes, a history of developmental regression, coarse facial features, seizures, and other types of movement disorders. Seizures and a history of developmental regression especially are important clinical denominators to consider an underlying inherited metabolic disorder in a patient with ataxia. Some of the inherited metabolic disorders have disease specific treatments to improve outcomes or prevent early death. Early diagnosis and treatment affect positive neurodevelopmental outcomes, so it is important to think of inherited metabolic disorders in the differential diagnosis of ataxia.
journal_name
Int J Mol Scijournal_title
International journal of molecular sciencesauthors
Silver G,Mercimek-Andrews Sdoi
10.3390/ijms21155519subject
Has Abstractpub_date
2020-08-01 00:00:00issue
15issn
1422-0067pii
ijms21155519journal_volume
21pub_type
杂志文章,评审abstract：:Nitrogen is an important macronutrient required for plant growth, and is a limiting factor for crop productivity. Improving the nitrogen use efficiency (NUE) is therefore crucial. At present, the NUE mechanism is unclear and information on the genes associated with NUE in soybeans is lacking. cystathionine beta syntha...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms17050620
更新日期：2016-04-26 00:00:00
abstract：:The physiological function of Arabidopsis thaliana universal stress protein (AtUSP) in plant has remained unclear. Thus, we report here the functional role of the Arabidopsis universal stress protein, AtUSP (At3g53990). To determine how AtUSP affects physiological responses towards cold stress, AtUSP overexpression (A...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms18122546
更新日期：2017-11-27 00:00:00
abstract：:Outbred female rats were exposed to inhalation of lead oxide nanoparticle aerosol produced right then and there at a concentration of 1.30 ± 0.10 mg/m3 during 5 days for 4 h a day in a nose-only setup. A control group of rats were sham-exposed in parallel under similar conditions. Even this short-time exposure of a re...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms21030690
更新日期：2020-01-21 00:00:00
abstract：:Members of the placenta-specific miRNA cluster C19MC, including miR-519d, are secreted by fetal trophoblast cells within extracellular vesicles (EVs). Trophoblast-derived EVs can be internalized by the autologous trophoblast and surrounding maternal immune cells, resulting in coordination of cellular responses. The st...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms21103458
更新日期：2020-05-14 00:00:00
abstract：:Phospholipidosis (PLD), the intracellular accumulation of phospholipids, is an adaptive response to toxic stimuli and serves as an important parameter in the biological assessment of compounds. Cationic amphiphilic drugs are the main inducers of PLD and may impair the function of alveolar macrophages. In vivo and in v...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms21218391
更新日期：2020-11-09 00:00:00
abstract：:Plants are constantly subjected to a variety of environmental stresses and have evolved regulatory responses to overcome unfavorable conditions that might reduce or adversely change a plant's growth or development. Among these, the regulated production of reactive oxygen species (ROS) as a signaling molecule occurs du...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms20061413
更新日期：2019-03-20 00:00:00
abstract：:miRNAs are small noncoding RNAs capable of regulating gene expression at the post-transcriptional level. A growing body of evidence demonstrated that let-7 family of miRNAs, as one of the highly conserved miRNAs, plays an important role in cell differentiation and development, as well as tumor suppressor function depe...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms151019119
更新日期：2014-10-21 00:00:00
abstract：:Oxylipins generated by the lipoxygenase (LOX) pathway play an important role in plant defense against biotic and abiotic stress. In chitosan-treated Adelostemma gracillimum seedlings, obvious accumulation of 9-LOX-derived oxylipins, namely 9,10,11-trihydroxy-12-octadecenoic acid, was detected. Using degenerate primers...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms13010540
更新日期：2012-01-01 00:00:00
abstract：:G protein-coupled receptors (GPCRs) are a large superfamily of cell-surface signaling proteins that bind extracellular ligands and transduce signals into cells via heterotrimeric G proteins. GPCRs are highly tractable drug targets. Aberrant expression of GPCRs and G proteins has been observed in various cancers and th...
journal_title：International journal of molecular sciences
pub_type： 杂志文章,评审
doi：10.3390/ijms17050707
更新日期：2016-05-11 00:00:00
abstract：:Of the multiple sources of reactive oxygen species (ROS) in the blood vessel, NADPH oxidases are the primary source. Whereas several studies have implicated NADPH oxidases in the initiation of atherosclerosis, their roles in disease progression are incompletely understood. Our objective was to determine the potential ...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms140817017
更新日期：2013-08-19 00:00:00
abstract：:A state of the art proteomic methodology using Matrix Assisted Laser Desorption/Ionization-Time of Flight (MALDI TOF) has been employed to characterize peptides modulated in the date palm stem subsequent to infestation with red palm weevil (RPW). Our analyses revealed 32 differentially expressed peptides associated wi...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms160819326
更新日期：2015-08-17 00:00:00
abstract：:The reaction product of nitric oxide and superoxide, peroxynitrite, is a potent biological oxidant. The most important oxidative protein modifications described for peroxynitrite are cysteine-thiol oxidation and tyrosine nitration. We have previously demonstrated that intrinsic heme-thiolate (P450)-dependent enzymatic...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms14047542
更新日期：2013-04-08 00:00:00
abstract：:Environmental or biomedical exposure to nanoparticles (NPs) can results in translocation and accumulation of NPs in the brain, which can lead to health-related problems. NPs have been shown to induce toxicity to neuronal cells through several direct mechanisms, but only a few studies have also explored the indirect ef...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms21197030
更新日期：2020-09-24 00:00:00
abstract：:We have generated the transgenic Tabaco plants expressing multiple monoclonal antibody (mAb) CO7-1A × BR55 by cross-pollinating with mAb CO17-1A and mAb BR55. We have demonstrated the anti-cancer effect of plant-derived multiple mAb CO17-1A × BR55. We find that co-treatment of colorectal mAbs (anti-epithelial cellular...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms151121105
更新日期：2014-11-14 00:00:00
abstract：:Sarcomere and cytoskeleton genes, or actomyosin genes, regulate cell biology including mechanical stress, cell motility, and cell division. While actomyosin genes are recurrently dysregulated in cancers, their oncogenic roles have not been examined in a lineage-specific fashion. In this report, we investigated dysregu...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms21228690
更新日期：2020-11-18 00:00:00
abstract：:Escherichia coli (E. coli) F18 is the main pathogen responsible for post-weaning diarrhea (PWD) in piglets. Resistance to E. coli F18 depends on the expression of the cognate receptors in the intestinal epithelial cells. However, the molecular mechanism of E. coli F18 resistance in weaned piglets remains unclear. Here...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms19113301
更新日期：2018-10-24 00:00:00
abstract：:The proportion of foodborne disease caused by pathogenic microorganisms is rising worldwide, with staphylococcal food poisoning being one of the main causes of this increase. Juglone is a plant-derived 1,4-naphthoquinone with confirmed antibacterial and antitumor activities. However, the specific mechanism underlying ...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms17060965
更新日期：2016-06-18 00:00:00
abstract：:Epigenetic factors are essentially involved in carcinogenesis, tumor promotion, and chemoresistance. Two epigenetic key players are miRNAs and histone deacetylases (HDACs). As previously shown by own theoretical databank analysis, the crosstalk between miRNAs and HDACs is relevant in different human chronic diseases a...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms19092781
更新日期：2018-09-15 00:00:00
abstract：:Factor binding IST-1 (FBI-1) plays an important role in oncogenic transformation and tumorigenesis. As FBI-1 is over-expressed in multiple human cancers, the regulation of itself would provide new effective options for cancer intervention. In this work, we aimed to study the role that EPAS-1 plays in regulating FBI-1....
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms150915689
更新日期：2014-09-04 00:00:00
abstract：:There is strong association between inflammatory processes and their main metabolic mediators, such as leptin, adiponectin secretion, and low/high-density lipoproteins, with the cancer risk and aggressive behavior of solid tumors. In this scenario, cancer cells (CCs) and cancer stem cells (CSCs) have important roles. ...
journal_title：International journal of molecular sciences
pub_type： 杂志文章,评审
doi：10.3390/ijms20102466
更新日期：2019-05-18 00:00:00
abstract：:We have recently found that diabetes-induced premature senescence of retinal endothelial cells is accompanied by NOX2-NADPH oxidase-induced increases in the ureohydrolase enzyme arginase 1 (A1). Here, we used genetic strategies to determine the specific involvement of A1 in diabetes-induced endothelial cell senescence...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms19041215
更新日期：2018-04-17 00:00:00
abstract：:The aim of the study was to investigate the effect of using direct electric current (DC) of 0, 200, and 400 mA for five minutes on the physiochemical properties, cytotoxicity, antibacterial, and antioxidant activity of sodium alginate hydrosols with different sodium chloride concentrations. The pH, oxidation-reduction...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms18030678
更新日期：2017-03-22 00:00:00
abstract：:Cover crops are key determinants of the ecological stability and sustainability of continuous cropping soils. However, their agro-ecological role in differentially reshaping the microbiome structure and functioning under a degraded agroecosystem remains poorly investigated. Therefore, structural and metabolic changes ...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms20112619
更新日期：2019-05-28 00:00:00
abstract：:Subcutaneous adipose tissue is a loose connective tissue specializing in the regulation of energy storage and metabolization. In domesticated pigs (Sus scrofa), the temporal development of subcutaneous adipose tissue is critical for meat production. However, the regulation of adipose tissue development remains unclear...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms19123892
更新日期：2018-12-05 00:00:00
abstract：:Diabetic nephropathy (DN) is the leading cause of end-stage renal disease in diabetes mellitus. Oxidative stress, insulin resistance and pro-inflammatory cytokines have been shown to play an important role in pathogeneses of renal damage on type 2 diabetes mellitus (DM). Inonotus obliquus (IO) is a white rot fungus th...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms17091535
更新日期：2016-09-13 00:00:00
abstract：:Oxathiapiprolin was developed as a specific plant pathogenic oomycete inhibitor, previously shown to have highly curative and protective activities against the pepper Phytophthora blight disease under field and greenhouse tests. Therefore, it was hypothesized that oxathiapiprolin might potentially activate the plant d...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms21041223
更新日期：2020-02-12 00:00:00
abstract：:Insulin-resistance is one of the main factors responsible for the onset and progression of Metabolic Syndrome (MetS). Among all polyphenols, the effects of flavonoids and their main food sources on insulin sensitivity have been widely evaluated in molecular and clinical studies. The aim of this review is to analyse th...
journal_title：International journal of molecular sciences
pub_type： 杂志文章,评审
doi：10.3390/ijms20092061
更新日期：2019-04-26 00:00:00
abstract：:The signaling function of the Na/K-ATPase has been established for 20 years and is widely accepted in the field, with many excellent reports and reviews not cited here. Even though there is debate about the underlying mechanism, the signaling function is unquestioned. This short review looks back at the evolution of N...
journal_title：International journal of molecular sciences
pub_type： 杂志文章,评审
doi：10.3390/ijms19092600
更新日期：2018-09-01 00:00:00
abstract：:Endocrine therapy is a key treatment strategy to control or eradicate hormone-responsive breast cancer. However, resistance to endocrine therapy leads to breast cancer relapse. The recent extension of adjuvant tamoxifen treatment up to 10 years actualizes the need for identifying biological markers that may be used to...
journal_title：International journal of molecular sciences
pub_type： 杂志文章,评审
doi：10.3390/ijms161024243
更新日期：2015-10-14 00:00:00
abstract：:Heterosis has been widely accepted as an effective strategy to increase yields in plant breeding. Notably, the chemical hybridization agent SQ-1 induces male sterility in wheat, representing a critical potential tool in hybrid seed production. However, the mechanisms underlying the male sterility induced by SQ-1 still...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms21072468
更新日期：2020-04-02 00:00:00
