Abstract:
:Niemann-Pick disease, type C1 (NPC1) is a fatal, autosomal recessive, neurodegenerative disorder caused by mutations in the NPC1 gene. As a result of the genetic defect, there is accumulation of unesterified cholesterol and sphingolipids in the late endosomal/lysosomal system causing both visceral and neurological defects. These manifest clinically as hepatosplenomegaly, liver dysfunction, and neurodegeneration. While significant progress has been made to better understand NPC1, the downstream effects of cholesterol storage and the major mechanisms that drive these pathologies remains less understood. In this study, it is sought to investigate free fatty acid levels in Npc1-/- mice with focus on the polyunsaturated ω-3 and ω-6 fatty acids. Since fatty acids are the main constituents of numerous lipids species, a discovery based lipidomic study of liver tissue in Npc1-/- mice is also performed. To this end, alterations in fatty acid synthesis, including the ω-3 and 6 fatty acids, are reported. Further, alterations in enzymes that regulate the synthesis of ω-3 and 6 fatty acids are reported. Analysis of the liver lipidome reveals alterations in both storage and membrane lipids including ceramides, fatty acids, phosphatidylcholamines, phosphatidylglycerols, phosphatidylethanolamines, sphingomyelins, and triacylglycerols in Npc1-/- mice at a late stage of disease.
journal_name
Proteomicsjournal_title
Proteomicsauthors
Pergande MR,Serna-Perez F,Mohsin SB,Hanek J,Cologna SMdoi
10.1002/pmic.201800285subject
Has Abstractpub_date
2019-09-01 00:00:00pages
e1800285issue
18eissn
1615-9853issn
1615-9861journal_volume
19pub_type
杂志文章相关文献
PROTEOMICS文献大全abstract::Long-lived conifer trees depend on both constitutive and induced defenses for resistance against a myriad of potential pathogens and herbivores. In species of spruce (Picea spp.), several of the late events of pathogen-, insect-, or elicitor-induced defense responses have previously been characterized at the anatomica...
journal_title:Proteomics
pub_type: 杂志文章
doi:10.1002/pmic.200800252
更新日期:2009-01-01 00:00:00
abstract::Glycosylphosphatidylinositol-anchored proteins (GPI-APs) are an important class of glycoproteins that are tethered to the surface of mammalian cells via the lipid GPI. GPI-APs have been implicated in many important cellular functions including cell adhesion, cell signaling, and immune regulation. Proteomic identificat...
journal_title:Proteomics
pub_type: 杂志文章
doi:10.1002/pmic.201400148
更新日期:2014-11-01 00:00:00
abstract::Acceleration of liver regeneration could be of great clinical benefit in various liver-associated diseases. However, at present little is known about therapeutic interventions to enhance this regenerative process. Our limited understanding and the complexity of the mechanisms involved have prevented the identification...
journal_title:Proteomics
pub_type: 杂志文章
doi:10.1002/pmic.200400913
更新日期:2005-01-01 00:00:00
abstract::An improved method for sample preparation for MALDI-MS and MS/MS using AnchorChip targets is presented. The method, termed the SMW method (sample, matrix wash), results in better sensitivity for peptide mass fingerprinting as well as for sequencing by MS/MS than previously published methods. The method allows up-conce...
journal_title:Proteomics
pub_type: 杂志文章
doi:10.1002/pmic.200600184
更新日期:2007-07-01 00:00:00
abstract::Proteome analysis by two-dimensional gel electrophoresis (2-DE) together with mass spectrometry was applied to screen acute phase response (APR)-related proteins with low molecular weight in loach skin following injury. Furthermore, Western blotting and function tests were applied to confirm the results obtained from ...
journal_title:Proteomics
pub_type: 杂志文章
doi:10.1002/pmic.200300864
更新日期:2004-12-01 00:00:00
abstract::In a comparative study, we investigated the influence of nine sample preparation workflows and seven different lysis buffers for qualitative and quantitative analysis of the human adipose tissue proteome. Adipose tissue is not just a fat depot but also an endocrine organ, which cross-talks with other tissue types and ...
journal_title:Proteomics
pub_type: 杂志文章
doi:10.1002/pmic.201500462
更新日期:2017-03-01 00:00:00
abstract::We bring a "spectrum" of classical data mining and statistical analysis methods to bear on discrimination of two groups of spectra from 24 diseased and 17 normal patients. Our primary goal is to accurately estimate the generalizability of this small dataset. After an aggressive preprocessing step that reduces consider...
journal_title:Proteomics
pub_type: 杂志文章
doi:10.1002/pmic.200300516
更新日期:2003-09-01 00:00:00
abstract::Plants exhibit resistance against incompatible pathogens, via localized and systemic responses as part of an integrated defense mechanism. To study the compatible and incompatible interactions between rice and bacteria, a proteomic approach was applied. Rice cv. Java 14 seedlings were inoculated with compatible (Xo743...
journal_title:Proteomics
pub_type: 杂志文章
doi:10.1002/pmic.200600470
更新日期:2006-11-01 00:00:00
abstract::We have used MALDI-MS imaging (MALDI-MSI) to monitor the time dependent appearance and loss of signals when tissue slices are brought rapidly to room temperature for short to medium periods of time. Sections from mouse brain were cut in a cryostat microtome, placed on a MALDI target and allowed to warm to room tempera...
journal_title:Proteomics
pub_type: 杂志文章
doi:10.1002/pmic.200800201
更新日期:2008-09-01 00:00:00
abstract::Eukaryotic cells plasma membranes are organized into microdomains of specialized function such as lipid rafts and caveolae, with a specific lipid composition highly enriched in cholesterol and glycosphingolipids. In addition to their role in regulating signal transduction, multiple functions have been proposed, such a...
journal_title:Proteomics
pub_type: 杂志文章
doi:10.1002/pmic.200500278
更新日期:2006-03-01 00:00:00
abstract::The main goal of many proteomics experiments is an accurate and rapid quantification and identification of regulated proteins in complex biological samples. The bottleneck in quantitative proteomics remains the availability of efficient software to evaluate and quantify the tremendous amount of mass spectral data acqu...
journal_title:Proteomics
pub_type: 杂志文章
doi:10.1002/pmic.200900174
更新日期:2010-01-01 00:00:00
abstract::As a consequence of their poor solubility during isoelectric focusing, integral membrane proteins are generally absent from two-dimensional gel proteome maps. In order to analyze the yeast plasma membrane proteome, a plasma membrane purification protocol was optimized in order to reduce contaminating membranes and cyt...
journal_title:Proteomics
pub_type: 杂志文章
doi:10.1002/1615-9861(200212)2:12<1706::AID-PROT1706>3
更新日期:2002-12-01 00:00:00
abstract::Pollution of soils by heavy metals is an ever-growing problem throughout the world, and is the result of human activities as well as geochemical weathering of rocks and other environmental causes such as volcanic eruptions, acid rain and continental dusts. Plants everywhere are continuously exposed to metal-contaminat...
journal_title:Proteomics
pub_type: 杂志文章,评审
doi:10.1002/pmic.200800935
更新日期:2009-05-01 00:00:00
abstract::Helicobacter pylori is one of the most prevalent human pathogens in the world and is the aetiological agent of gastritis, peptic ulcer disease and gastric malignancies. In addition H. pylori and other novel members of the genus are capable of successfully colonising the bile-rich niche of the upper intestine and are a...
journal_title:Proteomics
pub_type: 杂志文章
doi:10.1002/pmic.200390040
更新日期:2003-03-01 00:00:00
abstract::Pathogenic bacteria excrete a variety of virulence factors into extracellular medium and to the cell surface which have essential roles in the colonization and insurrection of the host cells, and thus reflect the degree of bacterial pathogenicity. For the exploration of virulence factors expressed in the secreted prot...
journal_title:Proteomics
pub_type: 杂志文章
doi:10.1002/pmic.200700965
更新日期:2008-06-01 00:00:00
abstract::Non-DNA microarrays, such as protein, peptide and small molecule microarrays, can potentially revolutionize the high-throughput screening tools currently used in basic and pharmaceutical research. However, fundamental obstacles remain that limit their rapid and widespread implementation as an alternative bioanalytical...
journal_title:Proteomics
pub_type: 杂志文章
doi:10.1002/pmic.200401078
更新日期:2005-05-01 00:00:00
abstract::Shewanella inhabit a wide variety of niches in nature and can utilize a broad spectrum of electron acceptors under anaerobic conditions. How they modulate their gene expression to adapt is poorly understood. ArcA, homologue of a global regulator controlling hundreds of genes involved in aerobic and anaerobic respirati...
journal_title:Proteomics
pub_type: 杂志文章
doi:10.1002/pmic.201100651
更新日期:2012-06-01 00:00:00
abstract::While metastasis is the primary cause of colorectal cancer (CRC) mortality, the molecular mechanisms underpinning it remains elusive. Metastasis is propagated through driver oncogene/suppressor gene mutations, accompanied by passenger mutations and underlying genomic instability. To understand cancer biology, a unifyi...
journal_title:Proteomics
pub_type: 杂志文章
doi:10.1002/pmic.201900026
更新日期:2019-11-01 00:00:00
abstract::Gliadins and glutenins are the main protein fractions present in wheat gluten. They are responsible for technological and nutritional quality of wheat based products. In particular, glutenins are mainly responsible for dough visco-elastic properties, whereas gliadins confer extensibility to dough and are the most impo...
journal_title:Proteomics
pub_type: 杂志文章
doi:10.1002/pmic.200500236
更新日期:2006-03-01 00:00:00
abstract::The skin provides protection against environmental stress. However, intrinsic and extrinsic aging causes significant alteration to skin structure and components, which subsequently impairs molecular characteristics and biochemical processes. Here, we have conducted an immunohistological investigation and established t...
journal_title:Proteomics
pub_type: 杂志文章
doi:10.1002/pmic.201600141
更新日期:2016-10-01 00:00:00
abstract::To gain a better understanding of salt stress responses in plants, we used a proteomic approach to investigate changes in rice (Oryza sativa) root plasma-membrane-associated proteins following treatment with 150 mmol/L NaCl. With or without a 48 h salt treatment, plasma membrane fractions from root tip cells of a salt...
journal_title:Proteomics
pub_type: 杂志文章
doi:10.1002/pmic.200800340
更新日期:2009-06-01 00:00:00
abstract::Many types of cells transit in vitro from a two- to a three-dimensional growth, when they are exposed to microgravity. The underlying mechanisms are not yet understood. Hence, we investigated the impact of microgravity on protein content and growth behavior. For this purpose, the human thyroid cancer cells FTC-133 wer...
journal_title:Proteomics
pub_type: 杂志文章
doi:10.1002/pmic.201500067
更新日期:2015-09-01 00:00:00
abstract::To better understand the mechanism underlying hepatocellular carcinoma (HCC) metastasis and to search for potential markers for HCC prognosis, differential proteome analysis on two HCC cell strains with high and low metastatic potentials, MHCC97-H and MHCC97-L, was conducted using two-dimensional (2-D) gel electrophor...
journal_title:Proteomics
pub_type: 杂志文章
doi:10.1002/pmic.200300653
更新日期:2004-04-01 00:00:00
abstract::Changing smooth muscle phenotype and abnormal cell proliferation are important features of vascular pathology, including the failure of saphenous vein bypass grafts. We have characterised and mapped protein expression in human saphenous vein medial smooth muscle, using two-dimensional (2-D) polyacrylamide gel electrop...
journal_title:Proteomics
pub_type: 杂志文章
doi:10.1002/1615-9861(200111)1:11<1405::AID-PROT1405>3
更新日期:2001-11-01 00:00:00
abstract::High-density lipoprotein (HDL) is the most abundant lipoprotein particle in the plasma and a negative risk factor of atherosclerosis. By using a proteomic approach it is possible to obtain detailed information about its protein content and protein modifications that may give new information about the physiological rol...
journal_title:Proteomics
pub_type: 杂志文章
doi:10.1002/pmic.200401010
更新日期:2005-04-01 00:00:00
abstract::The 26S proteasome is a multicatalytic protease complex that degrades ubiquitinated proteins in eukaryotic cells. It consists of a proteolytic core (the 20S proteasome) as well as regulatory particles, which contain six ATPase (Rpt) subunits involved in unfolding and translocation of substrates to the catalytic chambe...
journal_title:Proteomics
pub_type: 杂志文章
doi:10.1002/pmic.201300207
更新日期:2013-11-01 00:00:00
abstract::ProDaC (Proteomics Data Collection), a "Coordination Action" within the 6(th) EU framework programme, was created to support the collection, distribution and public availability of data from proteomics experiments. Within the consortium standards are created and maintained enabling an extensive data collection within ...
journal_title:Proteomics
pub_type: 杂志文章
doi:10.1002/pmic.200800732
更新日期:2009-01-01 00:00:00
abstract::Seed oil bodies (OBs) are intracellular particles storing lipids as food or biofuel reserves in oleaginous plants. Since Brassica napus OBs could be easily contaminated with protein bodies and/or myrosin cells, they must be purified step by step using floatation technique in order to remove non-specifically trapped pr...
journal_title:Proteomics
pub_type: 杂志文章
doi:10.1002/pmic.200800449
更新日期:2009-06-01 00:00:00
abstract::During the peri-implantation period, molecular signaling between embryo and endometrium (layer of tissue lining the uterus lumen) is supposed to be crucial for the maintenance of pregnancy. To investigate embryo-induced alterations in the proteome of bovine endometrium in the preattachment period (day 18), we used mon...
journal_title:Proteomics
pub_type: 杂志文章
doi:10.1002/pmic.200401242
更新日期:2005-07-01 00:00:00
abstract::Serum is an ideal biological sample that contains an archive of information due to the presence of a variety of proteins released by diseased tissue, and serum proteomics has gained considerable interest for the disease biomarker discovery. Easy accessibility and rapid protein changes in response to disease pathogenes...
journal_title:Proteomics
pub_type: 杂志文章,评审
doi:10.1002/pmic.201000460
更新日期:2011-06-01 00:00:00