Abstract:
UNLABELLED:Acute intermittent porphyria (AIP) is an autosomal dominant metabolic disorder caused by a deficiency of hepatic porphobilinogen deaminase (PBGD). The disease is characterized by life threatening acute neurovisceral attacks. The aim of this study was to identify metabolites secreted by the hepatocytes that reflect differential metabolic status in the liver and that may predict response to the acute attack treatment. Plasma vitamin D binding protein (VDBP) from a mouse model of AIP displayed an abnormal migration in 2D-electrophoresis that is efficiently recovered upon gene therapy leading to liver specific over-expression of the PBGD protein. The change in VDBP mobility results from a differential isoelectric point suggesting a post-translational modification that takes place preferably in the liver. Liquid chromatography-mass spectrometry (LC-MS) analysis of human samples before and after glycosidase treatment revealed glycosylated plasma VDBP specifically in patients with recurrent attacks of AIP. Glycosylated VDBP recovered normal values in three severely afflicted AIP patients submitted to therapeutic liver transplantation. Our findings suggest that post-translational modification of VDBP might be considered as a promising biomarker to study and monitor the liver metabolic status in patients with AIP. SIGNIFICANCE:We describe an increased glycosylation of VDBP in porphyric livers. Normal glycosylation was recovered upon liver gene therapy in a mouse model of porphyria or after liver transplantation in severely afflicted patients with AIP. Moreover, quantification of glycosylated VDBP by our ELISA immunoassay or LC-MS protocol in patients undergoing PBGD-gene therapy (www.aipgene.org) may be used as a marker indicating improvement or normalization of the patient's hepatic metabolism. This article is part of a Special Issue entitled: HUPO 2014.
journal_name
J Proteomicsjournal_title
Journal of proteomicsauthors
Serrano-Mendioroz I,Sampedro A,Mora MI,Mauleón I,Segura V,Enríquez de Salamanca R,Harper P,Sardh E,Corrales FJ,Fontanellas Adoi
10.1016/j.jprot.2015.05.004subject
Has Abstractpub_date
2015-09-08 00:00:00pages
377-85issue
Pt Beissn
1874-3919issn
1876-7737pii
S1874-3919(15)30001-4journal_volume
127pub_type
临床试验,杂志文章abstract::The biological functions of protein glycosylation have been increasingly recognized but not yet been very well understood, especially in lower organisms. Silkworm as a model lepidopteran insect and important economic insect, has been widely studied in life science, however, the current knowledge on the glycosylation s...
journal_title:Journal of proteomics
pub_type: 杂志文章
doi:10.1016/j.jprot.2020.103802
更新日期:2020-06-30 00:00:00
abstract:UNLABELLED:Increasing atmospheric carbon dioxide results in ocean acidification and warming, significantly impacting marine invertebrate larvae development. We investigated how ocean acidification in combination with warming affected D-veliger larvae of the Pacific oyster Crassostrea gigas. Larvae were reared for 40h u...
journal_title:Journal of proteomics
pub_type: 杂志文章
doi:10.1016/j.jprot.2015.12.001
更新日期:2016-03-01 00:00:00
abstract::High content omic techniques in combination with stable human in vitro cell culture systems have the potential to improve on current pre-clinical safety regimes by providing detailed mechanistic information of altered cellular processes. Here we investigated the added benefit of integrating transcriptomics, proteomics...
journal_title:Journal of proteomics
pub_type: 杂志文章
doi:10.1016/j.jprot.2012.11.022
更新日期:2013-02-21 00:00:00
abstract::Since its introduction mass spectrometry imaging (MSI) has proven to be a powerful tool for the localization of molecules in biological tissues. In drug discovery and development, understanding the distribution of both drug and its metabolites is of critical importance. Traditional methods suffer from a lack of spatia...
journal_title:Journal of proteomics
pub_type: 杂志文章,评审
doi:10.1016/j.jprot.2012.07.028
更新日期:2012-08-30 00:00:00
abstract:UNLABELLED:Marine bioadhesives have unmatched performances in wet environments, being an inspiration for biomedical applications. In sea urchins specialized adhesive organs, tube feet, mediate reversible adhesion, being composed by a disc, producing adhesive and de-adhesive secretions, and a motile stem. After tube foo...
journal_title:Journal of proteomics
pub_type: 杂志文章
doi:10.1016/j.jprot.2016.02.026
更新日期:2016-04-14 00:00:00
abstract::An automated image analysis system was used for protein quantification of 1862 human proteins in 47 cancer cell lines and 12 clinical cell samples using cell microarrays and immunohistochemistry. The analysis suggests that most proteins are expressed in a cell size dependent manner, and that normalization is required ...
journal_title:Journal of proteomics
pub_type: 杂志文章
doi:10.1016/j.jprot.2008.06.014
更新日期:2008-10-07 00:00:00
abstract::Tissues hypoxia caused by hemorrhage is a common complication in many clinical diseases. However, its pathological mechanism remains largely unknown. To partly address this issue, an ischemic-hypoxic rat model was established and the plasma proteomic and metabolic profiles were quantified and analyzed using TMT-based ...
journal_title:Journal of proteomics
pub_type: 杂志文章
doi:10.1016/j.jprot.2020.103924
更新日期:2020-09-30 00:00:00
abstract::Lignocellulosic plant biomass is the most abundant carbon source in the planet, which makes it a potential substrate for biorefinery. It consists of polysaccharides and other molecules with applications in pharmaceutical, food and feed, cosmetics, paper and textile industries. The exploitation of these resources requi...
journal_title:Journal of proteomics
pub_type: 杂志文章
doi:10.1016/j.jprot.2018.02.012
更新日期:2018-04-15 00:00:00
abstract:UNLABELLED:A literature survey covering the presence of bioactive peptides in plant-derived foodstuffs is presented. Examples are given of plant peptides associated with a beneficial effect on human health. The main bioactive effects of these peptides are defined and their mechanism of action described, when known. Cur...
journal_title:Journal of proteomics
pub_type: 杂志文章,评审
doi:10.1016/j.jprot.2016.03.048
更新日期:2016-09-16 00:00:00
abstract::In pre-weaning calves, both leucine and threonine play important roles in growth and muscle metabolism. In this study, metabolomics, proteomics and clinical chemistry were used to assess the effects of leucine and threonine supplementation added to milk replacer on 14 newborn Holstein male calves: 7 were fed a control...
journal_title:Journal of proteomics
pub_type: 杂志文章
doi:10.1016/j.jprot.2020.103677
更新日期:2020-03-30 00:00:00
abstract:UNLABELLED:The scorpion family Chaerilidae is phylogenetically differentiated from Buthidae. Their venom components are not known, and the evolution of the venom components is not well understood. Here, we performed a transcriptome analysis of the venom glands from two scorpion species, Chaerilus tricostatus and Chaeri...
journal_title:Journal of proteomics
pub_type: 杂志文章
doi:10.1016/j.jprot.2013.06.007
更新日期:2013-08-26 00:00:00
abstract::A large-scale proteomic approach was devised to advance the understanding of venom composition. Bothrops jararaca venom was fractionated by OFFGEL followed by chromatography, generating peptidic and proteic fractions. The latter was submitted to trypsin digestion. Both fractions were separately analyzed by reversed-ph...
journal_title:Journal of proteomics
pub_type: 杂志文章
doi:10.1016/j.jprot.2016.06.029
更新日期:2017-01-16 00:00:00
abstract:INTRODUCTION:Juvenile idiopathic arthritis (JIA) comprises a poorly understood group of chronic autoimmune diseases with variable clinical outcomes. We investigated whether the synovial fluid (SF) proteome could distinguish a subset of patients in whom disease extends to affect a large number of joints. METHODS:SF sam...
journal_title:Journal of proteomics
pub_type: 杂志文章
doi:10.1016/j.jprot.2012.06.024
更新日期:2012-09-18 00:00:00
abstract:UNLABELLED:Quercus suber L. is a forest tree with remarkable ecological, social and economic value in the southern Europe ecosystems. To circumvent the difficulties of breeding such long-lived species like Q. suber in a conventional fashion, clonal propagation of Q. suber elite trees can be carried out, although this p...
journal_title:Journal of proteomics
pub_type: 杂志文章
doi:10.1016/j.jprot.2013.06.006
更新日期:2013-11-20 00:00:00
abstract:UNLABELLED:In this study, a comparative proteomic analysis was employed to identify fuzz fiber initiation-related proteins in wild-type diploid cotton (Gossypium arboreum L.) and its fuzzless mutant. Temporal changes in global proteomes were examined using 2-DE at five developmental time points for fuzz fiber initiatio...
journal_title:Journal of proteomics
pub_type: 杂志文章
doi:10.1016/j.jprot.2013.02.020
更新日期:2013-04-26 00:00:00
abstract::Considering that proteins are usually engaged in complex and dynamic networks of interactions to exert their activity, a way to understand proteins' functions and the molecular mechanisms in which those proteins are involved, is by studying their interactome. In this sense, this tutorial presents a simple pipeline for...
journal_title:Journal of proteomics
pub_type: 杂志文章,评审
doi:10.1016/j.jprot.2017.04.011
更新日期:2018-01-16 00:00:00
abstract::Ubiquitylation is an elaborate post-translational modification involved in all biological processes. Its pleotropic effect is driven by the ability to form complex polyubiquitin chain architectures that can influence biological functions. In this study, we optimised sample preparation and chromatographic separation of...
journal_title:Journal of proteomics
pub_type: 杂志文章
doi:10.1016/j.jprot.2020.103963
更新日期:2020-10-30 00:00:00
abstract::The Estuarine stonefish (Synanceia horrida) is recognised as one of the most venomous fish species in the world but the overall venom composition has yet to be investigated using in-depth transcriptomic and proteomic methods. To date, known venom components are restricted to a hyaluronidase and a large, pore-forming t...
journal_title:Journal of proteomics
pub_type: 杂志文章
doi:10.1016/j.jprot.2019.04.002
更新日期:2019-06-15 00:00:00
abstract::Dystrobrevin is a dystrophin-related component of the dystrophin-associated protein complex (DAPC). Using alpha-dystrobrevin as indicator, we aimed to elucidate the interaction network of the DAPC with other proteins during apoptosis of promyelocytic HL-60 cells. The precise role(s) of DBs are not known, but we and ot...
journal_title:Journal of proteomics
pub_type: 杂志文章
doi:10.1016/j.jprot.2012.03.041
更新日期:2012-06-18 00:00:00
abstract::We carried out a comparative proteomic study to explore the molecular mechanisms that underlie the defense response of Douglas-fir (DF, Pseudotsuga menziesii) to laminated root rot, a disease caused by Phellinus sulphurascens. 2-DE was conducted on proteins extracted from roots of laboratory-grown, young DF seedlings ...
journal_title:Journal of proteomics
pub_type: 杂志文章
doi:10.1016/j.jprot.2008.06.004
更新日期:2008-10-07 00:00:00
abstract:UNLABELLED:Hg discharged into the environmental waters can generally be bioaccumulated, transformed and transmited by living organisms, thus resulting in the formation of Hg-toxicity food chains. The pathway and toxicology of food chain contaminated with environmental Hg are rarely revealed by proteomics. Here, we show...
journal_title:Journal of proteomics
pub_type: 杂志文章
doi:10.1016/j.jprot.2013.08.018
更新日期:2013-12-06 00:00:00
abstract::The present study describes the response of a bacterial strain, isolated from a hot spring in an area with the highest levels of natural radiation, under radium ((226)Ra) stress. The bacterium has been characterized as a novel and efficient radium biosorbent and identified as a variant of Serratia marcescens by bioche...
journal_title:Journal of proteomics
pub_type: 杂志文章
doi:10.1016/j.jprot.2012.05.020
更新日期:2012-08-03 00:00:00
abstract::Mass spectrometry is the technique of reference for the identification and quantification of proteins. Whereas ESI and MALDI ionization sources are inherently not quantitative being highly influenced by the chemical nature of the analyte and the matrix, ICP-MS uses a hard ionization source that destroys proteins into ...
journal_title:Journal of proteomics
pub_type: 杂志文章,评审
doi:10.1016/j.jprot.2018.11.010
更新日期:2019-04-30 00:00:00
abstract:UNLABELLED:The exoproteome of Leishmania infantum is composed of parasite derived proteins present in the extracellular environment. Although the exoproteome might have a significant role in the precocious steps of infection little is known concerning its composition. We developed an approach enabling the in vitro reco...
journal_title:Journal of proteomics
pub_type: 杂志文章
doi:10.1016/j.jprot.2013.03.012
更新日期:2013-06-12 00:00:00
abstract::The process of protein phosphorylation in cells is well studied in the context of a wide range of biologic functions such as signalling, cell cycle, cell growth and differentiation, and others. In contrast, little progress has been made in the investigation of protein phosphorylation specifically in blood. Here, we fo...
journal_title:Journal of proteomics
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1016/j.jprot.2012.02.015
更新日期:2012-12-05 00:00:00
abstract:UNLABELLED:Alterations in the sera proteins between patients with Primary Open-Angle Glaucoma (POAG), Pseudoexfoliation Glaucoma (PEXG), and healthy controls were identified through a proven approach utilizing equalization of high-abundance serum proteins with ProteoMiner™, two-dimensional fluorescent difference gel el...
journal_title:Journal of proteomics
pub_type: 临床试验,杂志文章
doi:10.1016/j.jprot.2013.12.006
更新日期:2014-02-26 00:00:00
abstract:UNLABELLED:High-throughput molecular analysis is challenging in a non-model species. Proteomics has a great potential to characterize non-model species. We went beyond the level of simply 'identifying' the proteins of interest during osmotic stress experiments in an allopolyploid crop (in casu banana) and focus on an i...
journal_title:Journal of proteomics
pub_type: 杂志文章
doi:10.1016/j.jprot.2015.01.014
更新日期:2015-04-24 00:00:00
abstract::Gluten proteins are major determinants of the bread making quality of wheat, but also of important wheat-related disorders, including coeliac disease (CD), and allergies. We carried out a proteomic study using the total grain proteins from two low-gliadin wheat lines, obtained by RNAi, and the untransformed wild type ...
journal_title:Journal of proteomics
pub_type: 杂志文章
doi:10.1016/j.jprot.2017.06.010
更新日期:2017-08-08 00:00:00
abstract:UNLABELLED:This study aimed at investigating the proteomic adaptation of Lactobacillus plantarum strains. Cultivation of L. plantarum strains under food-like conditions (wheat flour hydrolyzed, whey milk, tomato juice) affected some metabolic traits (e.g., consumption of carbohydrates and synthesis of organic acids) co...
journal_title:Journal of proteomics
pub_type: 杂志文章
doi:10.1016/j.jprot.2013.11.003
更新日期:2014-01-16 00:00:00
abstract:UNLABELLED:The venom proteome of the Malayan blue coral snake, Calliophis bivirgata flaviceps from west Malaysia was investigated by 1D-SDS-PAGE and shotgun-LCMS/MS. A total of 23 proteins belonging to 11 protein families were detected from the venom proteome. For the toxin proteins, the venom consists mainly of phosph...
journal_title:Journal of proteomics
pub_type: 杂志文章
doi:10.1016/j.jprot.2015.11.014
更新日期:2016-01-30 00:00:00