Abstract：
:The aim of this review article is to introduce the reader to the state-of-the-art of the contribution that proteomics and metabolomics sciences are currently providing for cystic fibrosis (CF) research: from the understanding of cystic fibrosis transmembrane conductance regulator (CFTR) biology to biomarker discovery for CF diagnosis. Our work particularly focuses on CFTR post-translational modifications and their role in cellular trafficking as well as on studies that allowed the identification of CFTR molecular interactors. We also show how metabolomics is currently helping biomarker discovery in CF. The most recent advances in these fields are covered by this review, as well as some considerations on possible future scenarios for new applications.
journal_name
Int J Mol Scijournal_title
International journal of molecular sciencesauthors
Liessi N,Pedemonte N,Armirotti A,Braccia Cdoi
10.3390/ijms21155439subject
Has Abstractpub_date
2020-07-30 00:00:00issue
15issn
1422-0067pii
ijms21155439journal_volume
21pub_type
杂志文章,评审abstract：:Toll-like receptor (TLR) 9 is an endosomal receptor recognizing bacterial DNA/CpG-containing oligodeoxynucleotides (CpG ODN). Blocking CpG ODN/TLR9 activity represents a strategy for therapeutic prevention of immune system overactivation. Herein, we report that a synthetic peptide (SP) representing the leucine-rich re...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms17020242
更新日期：2016-02-22 00:00:00
abstract：:Genetically modified vaccinia viruses (VACVs) have been shown to possess profound oncolytic capabilities. However, tumor cell resistance to VACVs may endanger broad clinical success. Using cell mass assays, viral replication studies, and fluorescence microscopy, we investigated primary resistance phenomena of cell lin...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms21207618
更新日期：2020-10-15 00:00:00
abstract：:N-acetylglutamate synthase deficiency (NAGSD) is an extremely rare urea cycle disorder (UCD) with few adult cases so far described. Diagnosis of late-onset presentations is difficult and delayed treatment may increase the risk of severe hyperammonemia. We describe a 52-year-old woman with recurrent headaches who exper...
journal_title：International journal of molecular sciences
pub_type： 杂志文章,评审
doi：10.3390/ijms19020345
更新日期：2018-01-24 00:00:00
abstract：:Verticillium dahliae (V. dahliae) infects roots and colonizes the vascular vessels of host plants, significantly reducing the economic yield of cotton and other crops. In this study, the protein VdTHI20, which is involved in the thiamine biosynthesis pathway, was characterized by knocking out the corresponding VdTHI20...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms21041378
更新日期：2020-02-18 00:00:00
abstract：:Following previous studies devoted to trans-Pt(3-af)2Cl2, in this paper, the molecular structure and intermolecular interactions of the title complex are compared with other cisplatin analogues of which the crystal structures are presented in the Cambridge Structural Database (CSD). Molecular Hirshfeld surface analysi...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms21062116
更新日期：2020-03-19 00:00:00
abstract：:The uptake and transport of iron (Fe) in plants are both important for plant growth and human health. However, little is known about the mechanism of Fe transport in plants, especially for crops. In the present study, the function of yellow stripe-like 13 (YSL13) in rice was analyzed. OsYSL13 was highly expressed in l...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms19113537
更新日期：2018-11-09 00:00:00
abstract：:Pancreatic ductal adenocarcinoma (PDAC) is an aggressive malignancy with increasing incidence and high mortality. Surgical resection is the only potentially curative treatment of patients with PDAC. Because of the late presentation of the disease, about 20 percent of patients are candidates for this treatment. The ave...
journal_title：International journal of molecular sciences
pub_type： 杂志文章,评审
doi：10.3390/ijms17060809
更新日期：2016-05-26 00:00:00
abstract：:Connexin hemichannels play an important role in the control of cellular signaling and behaviors. Given that lowering extracellular Ca2+, a condition that activates hemichannels, is a well-characterized stimulator of renin in juxtaglomerular cells, we, therefore, tested a potential implication of hemichannels in the re...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms21124462
更新日期：2020-06-23 00:00:00
abstract：:Calcific aortic valve disease (CAVD) is the result of maladaptive fibrocalcific processes leading to a progressive thickening and stiffening of aortic valve (AV) leaflets. CAVD is the most common cause of aortic stenosis (AS). At present, there is no effective pharmacotherapy in reducing CAVD progression; when CAVD be...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms21238917
更新日期：2020-11-24 00:00:00
abstract：:Immune modulation is a very modern medical field for targeting viral infections. In the race to develop the best immune modulator against viruses, curcumin, as a natural product, is inexpensive, without side effects, and can stimulate very well certain areas of the human immune system. As a bright yellow component of ...
journal_title：International journal of molecular sciences
pub_type： 杂志文章,评审
doi：10.3390/ijms21197150
更新日期：2020-09-28 00:00:00
abstract：:Parkinson's disease (PD) is a progressive neurological disorder marked by nigrostriatal dopaminergic degeneration. Evidence suggests that mitochondrial dysfunction may be linked to PD through a variety of different pathways, including free-radical generation and dysfunction of the mitochondrial Complex I activity. In ...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms13078722
更新日期：2012-01-01 00:00:00
abstract：:Sjögren's syndrome (SS) is a chronic autoimmune disease characterized by lymphocytic infiltration of salivary and lacrimal glands resulting in diminished production of saliva and tears. The pathophysiology of SS has not yet been fully deciphered. Classically it has been postulated that sicca symptoms in SS patients ar...
journal_title：International journal of molecular sciences
pub_type： 杂志文章,评审
doi：10.3390/ijms19113392
更新日期：2018-10-30 00:00:00
abstract：:For the past 20 years, we have witnessed an unprecedented and, indeed, rather miraculous event of how cell-penetrating peptides (CPPs), the naturally originated penetrating enhancers, help overcome the membrane barrier that has hindered the access of bio-macromolecular compounds such as genes and proteins into cells, ...
journal_title：International journal of molecular sciences
pub_type： 杂志文章,评审
doi：10.3390/ijms17111892
更新日期：2016-11-14 00:00:00
abstract：:The innate immune system has numerous mechanisms to fight against pathogens, including the formation of neutrophil extracellular traps (NETs). By spreading out chromatin, antimicrobial peptides and enzymes, neutrophils efficiently trap pathogens like bacteria and facilitate their elimination. During this process, high...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms19061679
更新日期：2018-06-05 00:00:00
abstract：:Calcium (Ca2+) plays an important role in regulating the differentiation and function of osteoclasts. Calcium oscillations (Ca oscillations) are well-known phenomena in receptor activator of nuclear factor kappa B ligand (RANKL)-induced osteoclastogenesis and bone resorption via calcineurin. Many modifiers are involve...
journal_title：International journal of molecular sciences
pub_type： 杂志文章,评审
doi：10.3390/ijms22010180
更新日期：2020-12-26 00:00:00
abstract：:Psoriasis vulgaris is an immune-mediated inflammatory skin disease. Although acitretin is a widely used synthetic retinoid for moderate to severe psoriasis, little is known about patients' genetics in response to this drug. In this study, 179 patients were enrolled in either the discovery set (13 patients) or replicat...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms18020295
更新日期：2017-01-29 00:00:00
abstract：:Macroautophagy, hereafter referred to as autophagy, is a bulk degradation process performed by lysosomes in which aggregated and altered proteins as well as dysfunctional organelles are decomposed. Autophagy is a basic cellular process that maintains homeostasis and is crucial for postmitotic neurons. Thus, impaired a...
journal_title：International journal of molecular sciences
pub_type： 杂志文章,评审
doi：10.3390/ijms161125990
更新日期：2015-11-09 00:00:00
abstract：:Drought stress is the major abiotic factor threatening maize (Zea mays L.) yield globally. Therefore, revealing the molecular mechanisms fundamental to drought tolerance in maize becomes imperative. Herein, we conducted a comprehensive comparative analysis of two maize inbred lines contrasting in drought stress tolera...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms19103225
更新日期：2018-10-18 00:00:00
abstract：:Little is known of the adaptive immune response to subarachnoid hemorrhage (SAH). This study was the first to investigate whether T cell receptor (TCR) immune repertoire may provide a better understanding of T cell immunology in delayed cerebral ischemia (DCI). We serially collected peripheral blood in five SAH patien...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms21093149
更新日期：2020-04-29 00:00:00
abstract：:Transcription factors play vital roles in the developmental processes of plants. The SQUAMOSA promoter binding protein (SBP) genes encode a family of plant-specific transcription factors and plays many crucial roles in plant development. In this study, 16 SBP-box gene family members were identified in Ziziphus jujuba ...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms18081734
更新日期：2017-08-15 00:00:00
abstract：:The relationship between molecularly imprinted polymer (MIP) morphology and template-rebinding over a series of warfarin-imprinted methacrylic acid co(ethylene dimethacrylate) polymers has been explored. Detailed investigations of the nature of template recognition revealed that an optimal template binding was obtain...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms14011207
更新日期：2013-01-09 00:00:00
abstract：:Understanding the kinetics of peptide self-assembly is important because of the involvement of peptide amyloid fibrils in several neurodegenerative diseases. In this paper, we have studied the dissolution kinetics of self-assembled model peptide fibrils after a dilution quench. Due to the low concentrations involved, ...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms21207671
更新日期：2020-10-16 00:00:00
abstract：:The aim of this study was to select effective and safe microbiocides for the disinfection and protection of historical wooden surfaces at the former Auschwitz II-Birkenau concentration and extermination camp. We tested seven active compounds against bacteria and moulds, of which didecyldimethylammonium chloride and N-...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms17081364
更新日期：2016-08-22 00:00:00
abstract：:Reactive oxygen species (ROS) are highly reactive signaling molecules that maintain redox homeostasis in mammalian cells. Dysregulation of redox homeostasis under pathological conditions results in excessive generation of ROS, culminating in oxidative stress and the associated oxidative damage of cellular components. ...
journal_title：International journal of molecular sciences
pub_type： 杂志文章,评审
doi：10.3390/ijms20153791
更新日期：2019-08-03 00:00:00
abstract：:Recent data show that cardiac hypertrophy contributes substantially to the overall heart failure burden. Mitochondrial dysfunction is a common feature of cardiac hypertrophy. Recent studies have reported that isosteviol inhibits myocardial ischemia-reperfusion injury in guinea pigs and H9c2 cells. This work investigat...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms21020682
更新日期：2020-01-20 00:00:00
abstract：:Oral squamous cell carcinoma (OSCC) is one of the leading presentations of head and neck cancer (HNC). The first part of this review will describe the highlights of the oral microbiome in health and normal development while demonstrating how both the oral and gut microbiome can map OSCC development, progression, treat...
journal_title：International journal of molecular sciences
pub_type： 杂志文章,评审
doi：10.3390/ijms21218061
更新日期：2020-10-29 00:00:00
abstract：:Polyphenol compositions and concentrations in skins and seeds of five muscadine grapes (cv. "Noble", "Alachua", "Carlos", "Fry", and "Granny Val") cultivated in the United States (Tallahassee-Florida, TA-FL) and South China (Nanning-Guangxi, NN-GX and Pu'er-Yunnan, PE-YN) were investigated, using ultra performance liq...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms18030631
更新日期：2017-03-14 00:00:00
abstract：:Despite intense research, few treatments are available for most neurological disorders. Demyelinating diseases are no exception. This is perhaps not surprising considering the multifactorial nature of these diseases, which involve complex interactions between immune system cells, glia and neurons. In the case of multi...
journal_title：International journal of molecular sciences
pub_type： 杂志文章,评审
doi：10.3390/ijms160921215
更新日期：2015-09-07 00:00:00
abstract：:We would like to submit the following correction to the published paper [1], the reason for this action is that the data in Table 3 were reanalyzed by one more accurate statistic method: On page 12, the sentence of paragraph three "OR and 95% CI were calculated by limited backward-LR (likelihood ratio) logistic regres...
journal_title：International journal of molecular sciences
pub_type： 已发布勘误
doi：10.3390/ijms18091899
更新日期：2017-09-04 00:00:00
abstract：:Interstitial telomeric sequences (ITSs) are short stretches of telomeric-like repeats (TTAGGG)n at nonterminal chromosomal sites. We previously demonstrated that, in the genomes of primates and rodents, ITSs were inserted during the repair of DNA double-strand breaks. These conclusions were derived from sequence compa...
journal_title：International journal of molecular sciences
pub_type： 杂志文章
doi：10.3390/ijms21082838
更新日期：2020-04-18 00:00:00
