Abstract:
:A decline in mitochondrial function has long been shown to exist in neurodegenerative disease. Whether this decline is a secondary consequence of other factors or whether it causes the eventual death of a cell is unknown. In this review, we will discuss some of the major evidence surrounding mitochondrial DNA mutations leading to mitochondrial dysfunction in neurodegenerative disease and discuss their possible role in neurodegeneration.
journal_name
Biochem Soc Transjournal_title
Biochemical Society transactionsauthors
Krishnan KJ,Reeve AK,Turnbull DMdoi
10.1042/BST0351232subject
Has Abstractpub_date
2007-11-01 00:00:00pages
1232-5issue
Pt 5eissn
0300-5127issn
1470-8752pii
BST0351232journal_volume
35pub_type
杂志文章,评审abstract::Reactive oxygen species (ROS) were originally thought to only be released by phagocytic cells during their role in host defence. It is now clear that ROS have a cell signalling role in many biological systems, both in animals and in plants. ROS induce programmed cell death or necrosis, induce or suppress the expressio...
journal_title:Biochemical Society transactions
pub_type: 杂志文章,评审
doi:10.1042/0300-5127:0290345
更新日期:2001-05-01 00:00:00
abstract::Mutations in MYO7A (myosin VIIa) cause Usher syndrome type 1B, a disorder involving profound congenital deafness and progressive blindness. In the retina, most MYO7A is localized in the apical region of the RPE (retinal pigmented epithelial) cells, and a small amount is associated with the ciliary and periciliary memb...
journal_title:Biochemical Society transactions
pub_type: 杂志文章,评审
doi:10.1042/BST0391207
更新日期:2011-10-01 00:00:00
abstract::Vitamin B12 (cobalamin) is a cobalt-containing modified tetrapyrrole that is an essential nutrient for higher animals. Its biosynthesis is restricted to certain bacteria and requires approximately 30 enzymatic steps for its complete de novo construction. Remarkably, two distinct biosynthetic pathways exist, which are ...
journal_title:Biochemical Society transactions
pub_type: 杂志文章,评审
doi:10.1042/BST20120066
更新日期:2012-06-01 00:00:00
abstract::Inflammation and dyslipidaemia both play important roles in the development of glomerular atherosclerosis in renal diseases. We have demonstrated that inflammatory mediators induced Scr (scavenger receptor) expression and the formation of foam cells, and that AP-1 (activator protein 1)/ets were necessary transcription...
journal_title:Biochemical Society transactions
pub_type: 杂志文章
doi:10.1042/bst0320088
更新日期:2004-02-01 00:00:00
abstract::The XMAP215 (Xenopus microtubule-associated protein 215) and CLASP [CLIP-170 (cytoskeletal linker protein 170) associated protein] microtubule plus end tracking families play central roles in the regulation of interphase microtubule dynamics and the proper formation of mitotic spindle architecture and flux. XMAP215 me...
journal_title:Biochemical Society transactions
pub_type: 杂志文章,评审
doi:10.1042/BST0371002
更新日期:2009-10-01 00:00:00
abstract::The completely sequenced genome of the cyanobacterium Synechocystis PCC 6803 contains several open reading frames, of which the deduced amino acid sequences show similarities to proteins known to be involved in FeS cluster synthesis of nitrogenase (Nif proteins) and other FeS proteins (Isc proteins). In this article, ...
journal_title:Biochemical Society transactions
pub_type: 杂志文章,评审
doi:10.1042/bst0290418
更新日期:2001-08-01 00:00:00
abstract::VEGFs (vascular endothelial growth factors) are master regulators of vascular development and of blood and lymphatic vessel function during health and disease in adults. This family of five mammalian ligands acts through three RTKs (receptor tyrosine kinases). In addition, co-receptors such as NRPs (neuropilins) assoc...
journal_title:Biochemical Society transactions
pub_type: 杂志文章,评审
doi:10.1042/BST20110689
更新日期:2012-02-01 00:00:00
abstract::The Wilms' tumour-suppressor gene (WT1), encodes a zinc-finger transcription factor that is critical for the development of several organs, including the kidneys, gonads and spleen. Despite its identification as a tumour suppressor that plays a crucial role in the formation of a paediatric malignancy of the kidneys (W...
journal_title:Biochemical Society transactions
pub_type: 杂志文章,评审
doi:10.1042/BST0360629
更新日期:2008-08-01 00:00:00
abstract::Protein aggregation is being found to be associated with an increasing number of human diseases. Aggregation can lead to a loss of function (lack of active protein) or to a toxic gain of function (cytotoxicity associated with protein aggregates). Although potentially harmful, protein sequences predisposed to aggregati...
journal_title:Biochemical Society transactions
pub_type: 杂志文章,评审
doi:10.1042/BST20120160
更新日期:2012-10-01 00:00:00
abstract::Post-translational modification (PTM) of histones has been investigated in eukaryotes for years, revealing its widespread occurrence and functional importance. Many PTMs affect chromatin folding and gene activity. Only recently the occurrence of such modifications has been recognized in bacteria. However, it is unclea...
journal_title:Biochemical Society transactions
pub_type: 杂志文章,评审
doi:10.1042/BST20180488
更新日期:2018-10-19 00:00:00
abstract::[Ca(2+)](i) (intracellular Ca(2+) concentration) oscillations play a central role in the activation of T-lymphocytes by antigen. Oscillations in T-cells are absolutely dependent on Ca(2+) influx through store-operated CRAC channels (Ca(2+)-release-activated Ca(2+) channels), and evidence suggests that they arise from ...
journal_title:Biochemical Society transactions
pub_type: 杂志文章,评审
doi:10.1042/bst0310925
更新日期:2003-10-01 00:00:00
abstract::Glyoxalase- and methylglyoxal-related research has required the development of quantitative and reliable techniques for the measurement of methylglyoxal-derived glycation adducts of protein and DNA. There are also other glycation adducts, oxidation adducts and nitration adducts of proteins and oxidation adducts of DNA...
journal_title:Biochemical Society transactions
pub_type: 杂志文章
doi:10.1042/BST20140019
更新日期:2014-04-01 00:00:00
abstract::Enzymes of the 1-deoxy-D-xylulose 5-phosphate/2-C-methylerythritol 4-phosphate (DOXP/MEP) pathway are targets for new herbicides and antibacterial drugs. Until now, no inhibitors for the DOXP synthase have been known of. We show that one of the breakdown products of the herbicide clomazone affects the DOXP synthase. O...
journal_title:Biochemical Society transactions
pub_type: 杂志文章
doi:
更新日期:2000-12-01 00:00:00
abstract::PTEN (phosphatase and tensin homologue deleted on chromosome 10) is an important tumour-suppressor gene that encodes a 3-phosphatase. The major substrate of PTEN is PIP(3) (phosphatidylinositol 3,4,5-trisphosphate) generated by the action of PI3Ks (phosphoinositide 3-kinases). Hereditary mutation of PTEN causes tumour...
journal_title:Biochemical Society transactions
pub_type: 杂志文章
doi:10.1042/BST0350172
更新日期:2007-04-01 00:00:00
abstract::It is now clear that NSPs (neutrophil serine proteases), including elastase, Pr3 (proteinase 3) and CatG (cathepsin G) are major pathogenic determinants in chronic inflammatory disorders of the lungs. Two unglycosylated natural protease inhibitors, SLPI (secretory leucocyte protease inhibitor) and elafin, and its prec...
journal_title:Biochemical Society transactions
pub_type: 杂志文章,评审
doi:10.1042/BST0391441
更新日期:2011-10-01 00:00:00
abstract::Luteinizing hormone (LH) interacts with its plasma membrane receptor to activate the formation of cyclic AMP via the regulatory GTP binding protein (Gs). This is followed by a desensitization of that same hormonal response which is caused by an uncoupling of the LH receptor from Gs. The coupling between Gs and the ade...
journal_title:Biochemical Society transactions
pub_type: 杂志文章,评审
doi:10.1042/bst0170633
更新日期:1989-08-01 00:00:00
abstract::Virtually all the biological processes that occur inside or outside cells are mediated by protein-protein interactions (PPIs). Hence, the charting and description of the PPI network, initially in organisms, the interactome, but more recently in specific tissues, is essential to fully understand cellular processes both...
journal_title:Biochemical Society transactions
pub_type: 杂志文章,评审
doi:10.1042/BST20150001
更新日期:2016-06-15 00:00:00
abstract::Glycogen synthase kinase-3 is an unusual protein serine/threonine kinase that, unlike most of its 500-odd relatives in the genome, is active under resting conditions and is inactivated upon cell stimulation. The two mammalian isoforms, GSK-3alpha and beta, play largely overlapping roles and have been implicated in a v...
journal_title:Biochemical Society transactions
pub_type: 杂志文章,评审
doi:10.1042/BST0320803
更新日期:2004-11-01 00:00:00
abstract::Other than signalling receptors sustaining leucocyte recruitment during inflammatory reactions, the chemokine system includes 'silent' receptors with distinct specificity and tissue distribution. The best-characterized molecule of this subgroup is the CC chemokine receptor D6, which binds most inflammatory CC chemokin...
journal_title:Biochemical Society transactions
pub_type: 杂志文章
doi:10.1042/BST0341014
更新日期:2006-12-01 00:00:00
abstract::PLUNC (palate, lung and nasal epithelium clone) proteins make up the largest branch of the BPI (bactericidal/permeability-increasing protein)/LBP (lipopolysaccharide-binding protein) family of lipid-transfer proteins. PLUNCs make up one of the most rapidly evolving mammalian protein families and exhibit low levels of ...
journal_title:Biochemical Society transactions
pub_type: 杂志文章,评审
doi:10.1042/BST0390961
更新日期:2011-08-01 00:00:00
abstract::From a random transposon mutagenesis experiment, using Petunia line W138, a seed-specific linoleic acid mutant was isolated. The tagged gene was cloned and identified as a microsomal Delta(12) desaturase. Expression of the gene, however, was constitutive and not, as might have been expected, seed-specific. Moreover, s...
journal_title:Biochemical Society transactions
pub_type: 杂志文章
doi:
更新日期:2000-12-01 00:00:00
abstract::Allostery is a biological phenomenon of critical importance in metabolic regulation and cell signalling. The fundamental premise of classical models that describe allostery is that structure mediates 'action at a distance'. Recently, this paradigm has been challenged by the enrichment of IDPs (intrinsically disordered...
journal_title:Biochemical Society transactions
pub_type: 杂志文章,评审
doi:10.1042/BST20120163
更新日期:2012-10-01 00:00:00
abstract::This article reviews the progress of a personal endeavour to develop chromatography as a quantitative procedure for the determination of reaction stoichiometries and equilibrium constants governing protein interactions. As well as affording insight into an aspect of chromatography with which many protein chemists are ...
journal_title:Biochemical Society transactions
pub_type: 杂志文章,评审
doi:10.1042/bst0311010
更新日期:2003-10-01 00:00:00
abstract::Saccharomyces cerevisiae cells grown in glucose have larger average size than cells grown in ethanol. Besides, yeast must reach a carbon source-modulated critical cell size in order to enter S phase at Start. This control is of outmost physiological relevance, since it allows us to coordinate cell growth with cell cyc...
journal_title:Biochemical Society transactions
pub_type: 杂志文章,评审
doi:10.1042/BST0330294
更新日期:2005-02-01 00:00:00
abstract::Exosomes are small extracellular vesicles which stem from endosomes fusing with the plasma membrane; they contain lipids, proteins and RNAs that are able to modify receiving cells. Functioning of the brain relies on synapses, and certain patterns of synaptic activity can change the strength of responses at sparse grou...
journal_title:Biochemical Society transactions
pub_type: 杂志文章,评审
doi:10.1042/BST20120266
更新日期:2013-02-01 00:00:00
abstract::Ligand-gated ion channels function as rapid signal transducers, converting chemical signals (in the form of neurotransmitters) into electrical signals in the postsynaptic neuron. This is achieved by the recognition of neurotransmitter at its specific-binding sites, which then triggers the opening of an ion channel ('g...
journal_title:Biochemical Society transactions
pub_type: 杂志文章,评审
doi:10.1042/BST0320540
更新日期:2004-06-01 00:00:00
abstract::CYP74C3 (cytochrome P450 subfamily 74C3), an HPL (hydroperoxide lyase) from Medicago truncatula (barrel medic), and CYP74A1, an AOS (allene oxide synthase) from Arabidopsis thaliana, are key membrane-associated P450 enzymes in plant oxylipin metabolism. Both recombinant detergent-free enzymes are monomeric proteins wi...
journal_title:Biochemical Society transactions
pub_type: 杂志文章
doi:10.1042/BST0341223
更新日期:2006-12-01 00:00:00
abstract::Single-molecule fluorescence methods, particularly single-molecule FRET (fluorescence resonance energy transfer), have provided novel insights into the structure, interactions and dynamics of biological systems. ALEX (alternating-laser excitation) spectroscopy is a new method that extends single-molecule FRET by provi...
journal_title:Biochemical Society transactions
pub_type: 杂志文章
doi:10.1042/BST0360738
更新日期:2008-08-01 00:00:00
abstract::The past two decades in research has revealed the importance of leucine-rich repeat kinase 2 (LRRK2) in both monogenic and sporadic forms of Parkinson's disease (PD). In families, mutations in LRRK2 can cause PD with age-dependent but variable penetrance and genome-wide association studies have found variants of the g...
journal_title:Biochemical Society transactions
pub_type: 杂志文章,评审
doi:10.1042/BST20180462
更新日期:2019-04-30 00:00:00
abstract::The collagens of vertebrates may be divided into three groups according to chain size and whether or not the helical domains are continuous. Present evidence suggests that, at least within one of these groups, similarity between collagens is reflected in the organization of the genes that encode them. Early evidence s...
journal_title:Biochemical Society transactions
pub_type: 杂志文章,评审
doi:10.1042/bst0160661
更新日期:1988-10-01 00:00:00