Abstract:
:Mitochondrial dysfunction causes a range of early-onset neurological diseases and contributes to neurodegenerative conditions. The mechanisms of neurological damage however are poorly understood, as accessing relevant tissue from patients is difficult, and appropriate models are limited. Hence, we assessed mitochondrial function in neurologically relevant primary cell lines from a CI (complex I) deficient Ndufs4 KO (knockout) mouse (Ndufs4fky/fky) modelling aspects of the mitochondrial disease LS (Leigh syndrome), as well as MEFs (mouse embryonic fibroblasts). Although CI structure and function were compromised in all Ndufs4fky/fky cell types, the mitochondrial membrane potential was selectively impaired in the MEFs, correlating with decreased CI-dependent ATP synthesis. In addition, increased ROS (reactive oxygen species) generation and altered sensitivity to cell death were only observed in Ndufs4fky/fky primary MEFs. In contrast, Ndufs4fky/fky primary isocortical neurons and primary isocortical astrocytes displayed only impaired ATP generation without mitochondrial membrane potential changes. Therefore the neurological dysfunction in the Ndufs4fky/fky mouse may partly originate from a more severe ATP depletion in neurons and astrocytes, even at the expense of maintaining the mitochondrial membrane potential. This may provide protection from cell death, but would ultimately compromise cell functionality in neurons and astrocytes. Furthermore, RET (reverse electron transfer) from complex II to CI appears more prominent in neurons than MEFs or astrocytes, and is attenuated in Ndufs4fky/fky cells.
journal_name
Biosci Repjournal_title
Bioscience reportsauthors
Bird MJ,Wijeyeratne XW,Komen JC,Laskowski A,Ryan MT,Thorburn DR,Frazier AEdoi
10.1042/BSR20140151subject
Has Abstractpub_date
2014-11-21 00:00:00pages
e00151issue
6eissn
0144-8463issn
1573-4935pii
BSR20140151journal_volume
34pub_type
杂志文章abstract::The present study investigated the effects of Isorhamnetin on two types of prostate cancer cells (androgen-independent and androgen-dependent) and explored its possible mechanisms underlying such effects. Treatment with Isorhamnetin significantly inhibited cell growth and induced lactate dehydrogenase (LDH) release of...
journal_title:Bioscience reports
pub_type: 杂志文章
doi:10.1042/BSR20192826
更新日期:2020-03-27 00:00:00
abstract::Acquired resistance of afatinib is a significant challenge for non-small cell lung cancer (NSCLC) therapy and the mechanisms remain unclear. Aberrant activation of epidermal growth factor receptor (EGFR)-dependent downstream pathways, especially phosphatidylinositol-3-kinases/protein kinase B (PI3K/Akt) signaling path...
journal_title:Bioscience reports
pub_type: 杂志文章
doi:10.1042/BSR20181693
更新日期:2018-12-11 00:00:00
abstract::We have investigated the intramembranal ion traffic in apoptotic 3T6 cells in culture. Apoptosis was induced by various treatments, such as serum deprivation, high density growth and hydrogen peroxide at subnecrotic doses. Cell death was assessed by nucleosomal DNA fragmentation, single cell electrophoresis, immunoflu...
journal_title:Bioscience reports
pub_type: 杂志文章
doi:10.1023/a:1027364308147
更新日期:1997-12-01 00:00:00
abstract::A riboflavin carrier protein has been purified from human pregnancy and umbilical cord sera by affinity chromatography and fast protein liquid chromatography. This protein has a similar molecular weight to the chicken egg riboflavin carrier protein and shares other physicochemical properties, such as pI and riboflavin...
journal_title:Bioscience reports
pub_type: 杂志文章
doi:10.1007/BF01119773
更新日期:1987-07-01 00:00:00
abstract::Isoliquiritigenin (ISL), a natural product isolated from licorice root, exhibits anti-gastric cancer effects. However, applications of ISL are still limited in clinical practice due to its poor bioavailability. To discovery of more effective anti-gastric cancer agents based on ISL, aldol condensation reaction was appl...
journal_title:Bioscience reports
pub_type: 杂志文章
doi:10.1042/BSR20201199
更新日期:2020-06-26 00:00:00
abstract::The effect of carbamylcholine and the calcium ionophore A23187 on catecholamine release and intracellular free calcium, [Ca2+]i, in bovine adrenal chromaffin cells was determined. At 10(-4) M carbamylcholine maximal release occurred with an accompanying increase in [Ca2+]i from a basal level of 168 nM to less than 300...
journal_title:Bioscience reports
pub_type: 杂志文章
doi:10.1007/BF01121918
更新日期:1984-07-01 00:00:00
abstract::The sodium contents of beta-cell-rich pancreatic islets from ob/ob-mice were measured with an integrating flame photometer. After washing to an apparent steady state with different types of ice-cold media, islets incubated in the absence of glucose contained 79-108 mmol sodium kg-1 dry weight. Exposure to glucose resu...
journal_title:Bioscience reports
pub_type: 杂志文章
doi:10.1007/BF01114973
更新日期:1986-11-01 00:00:00
abstract::eNOS (endothelial nitric oxide synthase) contains a MAPK (mitogen-activated protein kinase)-binding site associated with a major eNOS control element. Purified ERK (extracellular-signal-regulated kinase) phosphorylates eNOS with a stoichiometry of 2-3 phosphates per eNOS monomer. Phosphorylation decreases NO synthesis...
journal_title:Bioscience reports
pub_type: 杂志文章
doi:10.1042/BSR20140015
更新日期:2014-09-17 00:00:00
abstract::Maclurin, the potent non-specific blood-group hemagglutinin present in extracts of Maclura pomifera, has been purified by a new biospecific affinity-chromatographic procedure. Additional studies have indicated that this hemagglutinin occurs as five closely related tetrameric protein isoforms derived from two non-coval...
journal_title:Bioscience reports
pub_type: 杂志文章
doi:10.1007/BF01136724
更新日期:1982-04-01 00:00:00
abstract::The 7-nitro-2,1,3-nitrobenzoxadiazole (NBD) derivatives are a series of compounds containing the NBD scaffold that are not glutathione (GSH) peptidomimetics, and result in a strong inhibition of glutathione S-transferases (GSTs). Growing evidences highlight their pivotal roles and outstanding anticancer activity in di...
journal_title:Bioscience reports
pub_type: 杂志文章,评审
doi:10.1042/BSR20171440
更新日期:2018-02-13 00:00:00
abstract:OBJECTIVE:The current study aimed to investigate the functional roles and clinical significance of microRNA-148a (miR-148a) in the progression of oral squamous cell carcinoma (OSCC). METHODS:Relative expression of miR-148a in OSCC cells and tissues were detected using quantitative real-time polymerase chain reaction (...
journal_title:Bioscience reports
pub_type: 杂志文章
doi:10.1042/BSR20182458
更新日期:2020-04-30 00:00:00
abstract::microRNAs (miRNAs) have been found to affect various cancers, and expression of numerous miRNAs is revealed in glioma. However, the role of microRNA-30b-3p (miR-30b-3p) in glioma remains elusive. Therefore, the present study aims to explore the specific mechanism by which miR-30b-3p influence the development of glioma...
journal_title:Bioscience reports
pub_type: 杂志文章
doi:10.1042/BSR20182226
更新日期:2019-08-13 00:00:00
abstract::Neuroblastoma, which accounts for approximately 10% of all pediatric cancer-related deaths, has become a therapeutic challenge and global burden attributed to poor outcomes and mortality rates of its high-risk form. Previous genome-wide association studies (GWASs) identified the LINC00673 rs11655237 C>T polymorphism t...
journal_title:Bioscience reports
pub_type: 杂志文章
doi:10.1042/BSR20171667
更新日期:2018-02-08 00:00:00
abstract::Imipramine and clorgyline, at concentrations of 0.002 M, inhibit the respiration of brain tissue by 82 and 71 per cent respectively, while chloropromazine and tranylcypromine, at concentrations of 0.01 M, inhibit it about 25 per cent. Deprenyl and amphetamine at a concentration of 0.002 M inhibit brain tissue respirat...
journal_title:Bioscience reports
pub_type: 杂志文章
doi:10.1007/BF01118600
更新日期:1991-02-01 00:00:00
abstract::Polycationic amino acids induce the leakage and fusion of liposomes containing anionic lipids. We have investigated the nature and extent of the changes in membrane physical properties caused by these polypeptides which could result in the observed membrane destabilization. We found that in the range of pH 5 to pH 7 b...
journal_title:Bioscience reports
pub_type: 杂志文章
doi:10.1007/BF01207455
更新日期:1995-06-01 00:00:00
abstract::TRα1 (thyroid hormone receptor α1) is well recognized for its importance in brain development. However, due to the difficulties in predicting TREs (thyroid hormone response elements) in silico and the lack of suitable antibodies against TRα1 for ChIP (chromatin immunoprecipitation), only a few direct TRα1 target genes...
journal_title:Bioscience reports
pub_type: 杂志文章
doi:10.1042/BSR20120124
更新日期:2013-03-13 00:00:00
abstract::Retinitis pigmentosa (RP) is a group of hereditary, degenerative retinal disorders characterized by progressive retinal dysfunction, outer retina cell loss, and retinal tissue atrophy. It eventually leads to tunnel vision and legal, or total blindness. Here we aimed to reveal the causal gene and mutation contributing ...
journal_title:Bioscience reports
pub_type: 杂志文章
doi:10.1042/BSR20171300
更新日期:2018-04-27 00:00:00
abstract:OBJECTIVES:To investigate the association between dectin-1 gene single nucleotide polymorphisms (SNPs) and susceptibility to fungal infection (FI). METHODS:Databases were searched electronically and manually to identify case-control studies concerning dectin-1 SNPs and FI, which were published up to 12 November 2018. ...
journal_title:Bioscience reports
pub_type: 杂志文章,meta分析
doi:10.1042/BSR20191519
更新日期:2019-11-29 00:00:00
abstract::Background: Endometrial carcinoma (EC) still threatens the health of women. Thus, to explore how long intergenic non-protein coding RNA 01220 regulates the development of EC.Methods: Whole genome expression profile data of EC and paracancerous tissues in TCGA database were downloaded. LINC01220 expression in EC and pa...
journal_title:Bioscience reports
pub_type: 杂志文章
doi:10.1042/BSR20181794
更新日期:2019-07-25 00:00:00
abstract::LGMD (limb-girdle muscular dystrophy) and CMD (congenital muscular dystrophy) are two common forms of neuromuscular disorders which are distinguishable by their age of onset but with probably a similar underlying pathway. In the present study, we report immunohistochemical, Western-blot and genetic analyses in a large...
journal_title:Bioscience reports
pub_type: 杂志文章
doi:10.1042/BSR20100026
更新日期:2011-04-01 00:00:00
abstract:BACKGROUND/AIMS:MicroRNAs (miRNAs) are involved in the pathogenesis of osteoarthritis (OA). The present study aimed to investigate the potential function of miR-122 in the development of OA and its potential molecular mechanisms. METHODS:The expression of miR-122, silent information regulator 1 (SIRT1), collagen II, a...
journal_title:Bioscience reports
pub_type: 杂志文章
doi:10.1042/BSR20191908
更新日期:2020-06-26 00:00:00
abstract::The association between dietary protein intake and ovarian cancer had been inconsistent in the previous epidemiological studies. The aim of the present study was to identify and synthesize all citations evaluating the relationship on ovarian cancer with protein intake. The search included PubMed, Embase, and Web of Sc...
journal_title:Bioscience reports
pub_type: 杂志文章,meta分析
doi:10.1042/BSR20181857
更新日期:2018-12-11 00:00:00
abstract::Gold thioglucose and gold sodium thiomalate were shown to be potent activators of latent human leukocyte collagenase. No activation by auranofin was noted. The activation may proceed through the action of gold on the essential sulfhydryl groups of latent enzyme and, thereby, mimick the action of the known organomercur...
journal_title:Bioscience reports
pub_type: 杂志文章
doi:10.1007/BF01122130
更新日期:1987-12-01 00:00:00
abstract::Background: There is a clinical need for the use of engineered adipose tissue in place of surgical reconstruction. We previously found that the external volume expansion (EVE) device increased special cell clusters in well-vascularized connective stroma during adipose regeneration. However, the origin of these cell cl...
journal_title:Bioscience reports
pub_type: 杂志文章
doi:10.1042/BSR20181776
更新日期:2019-02-26 00:00:00
abstract:AIM:The purpose of the present study was to explore the function and mechanism of tensin 1 (TNS1) in non-small cell lung cancer (NSCLC) progression. METHODS:The expression of TNS1 in NSCLC cells and tissues was assessed by RT-PCR and Western blot. Besides, Kaplan-Meier survival analysis was recruited to explore the as...
journal_title:Bioscience reports
pub_type: 杂志文章
doi:10.1042/BSR20201539
更新日期:2021-01-29 00:00:00
abstract::Extracorporeal pulsed electromagnetic field (PEMF) has shown the ability to regenerate tissue by promoting cell proliferation. In the present study, we investigated for the first time whether PEMF treatment could improve the myocardial ischaemia/reperfusion (I/R) injury and uncovered its underlying mechanisms.In our s...
journal_title:Bioscience reports
pub_type: 杂志文章
doi:10.1042/BSR20160082
更新日期:2016-12-05 00:00:00
abstract::The cDNA encoding about half of an antigenic non-surface schistosome parasite protein of Mr 97 K has recently been cloned and sequenced (Lanar, Pearce, James and Sher (1986) Science 234:593-596). Analysis of this sequence, together with the properties of the native protein, reveals that this protein is paramyosin, the...
journal_title:Bioscience reports
pub_type: 杂志文章
doi:10.1007/BF01122722
更新日期:1987-01-01 00:00:00
abstract::The ways in which certain inorganic elements are used in essential roles within biological systems generally are described. The two roles stressed are (1) electrolytic charge movements and (2) electronic charge movement. The value of helical proteins inside and outside membranes as mechanical coupling devices is analy...
journal_title:Bioscience reports
pub_type: 杂志文章
doi:10.1007/BF01117344
更新日期:1988-12-01 00:00:00
abstract::Rodney Porter's separation of antibody molecules into Fab and Fc fragments engendered the notion that a single antibody polypeptide chain might be coded by two or more genes. This concept profoundly influenced the development of molecular immunology over the past 25 years. Our current knowledge of antibody gene organi...
journal_title:Bioscience reports
pub_type: 杂志文章
doi:10.1007/BF01119901
更新日期:1985-10-01 00:00:00
abstract::Erythrocytes suspended in a medium of low ionic strength lyse under the effect of an exponential electrical pulse. The percentage of haemolysed cells decreases several-fold in the presence of divalent cations. The protective action of the ions studied increases in the following order: Ca++, Mg++, Zn++. It is assumed t...
journal_title:Bioscience reports
pub_type: 杂志文章
doi:10.1007/BF01121639
更新日期:1988-10-01 00:00:00