Huntington's disease and mitochondrial alterations: emphasis on experimental models.

Abstract:

:Huntington's disease (HD) is an inheritable neurological disorder coursing with degeneration of basal ganglia and producing chorea and dementia. One common factor accounting for neurodegeneration in this disorder is mitochondrial deterioration at both morphologic and functional levels. The development of experimental models in animals or cell preparations to resemble pathologic and pathogenic conditions of this disorder has served for more than four decades to describe part of the mechanistic alterations that could be occurring in mitochondria of HD patients, and the subsequent design of therapeutic alternatives where mitochondrial alterations are the primary target. In this minireview we describe some of the most relevant studies at the experimental level, giving support to the hypothesis that mitochondria play a central role in HD pathogenesis.

journal_name

J Bioenerg Biomembr

authors

Pérez-De la Cruz V,Carrillo-Mora P,Santamaría A

doi

10.1007/s10863-010-9289-4

subject

Has Abstract

pub_date

2010-06-01 00:00:00

pages

207-15

issue

3

eissn

0145-479X

issn

1573-6881

journal_volume

42

pub_type

杂志文章
  • A translational study "case report" on the small molecule "energy blocker" 3-bromopyruvate (3BP) as a potent anticancer agent: from bench side to bedside.

    abstract::The small alkylating molecule, 3-bromopyruvate (3BP), is a potent and specific anticancer agent. 3BP is different in its action from most currently available chemo-drugs. Thus, 3BP targets cancer cells' energy metabolism, both its high glycolysis ("Warburg Effect") and mitochondrial oxidative phosphorylation. This inh...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1007/s10863-012-9417-4

    authors: Ko YH,Verhoeven HA,Lee MJ,Corbin DJ,Vogl TJ,Pedersen PL

    更新日期:2012-02-01 00:00:00

  • α-Lipoic acid ameliorates impaired glucose uptake in LYRM1 overexpressing 3T3-L1 adipocytes through the IRS-1/Akt signaling pathway.

    abstract::Overexpression of the Homo sapiens LYR motif containing 1 (LYRM1) causes mitochondrial dysfunction and induces insulin resistance in 3T3-L1 adipocytes. α-Lipoic acid (α-LA), a dithiol compound with antioxidant properties, improves glucose transport and utilization in 3T3-L1 adipocytes. The aim of this study was to inv...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1007/s10863-012-9460-1

    authors: Qin ZY,Zhang M,Guo XR,Wang YM,Zhu GZ,Ni YH,Zhao YP,Qiu J,Kou CZ,Qin R,Cao XG

    更新日期:2012-10-01 00:00:00

  • Conformational changes in the Escherichia coli ATP synthase b-dimer upon binding to F(1)-ATPase.

    abstract::Conformational changes within the subunit b-dimer of the E. coli ATP synthase occur upon binding to the F(1) sector. ESR spectra of spin-labeled b at room temperature indicated a pivotal point in the b-structure at residue 62. Spectra of frozen b +/- F(1) and calculated interspin distances suggested that where contact...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1007/s10863-008-9189-z

    authors: Zaida TM,Hornung T,Volkov OA,Hoffman AD,Pandey SJ,Wise JG,Vogel PD

    更新日期:2008-12-01 00:00:00

  • Structure, function and regulation of the tricarboxylate transport protein from rat liver mitochondria.

    abstract::Recent progress is summarized on the structure, function, and regulation of the tricarboxylate (i.e., citrate) transport protein (CTP) from the rat liver mitochondrial inner membrane. The transporter has been purified and its reconstituted function characterized. A cDNA clone encoding the CTP has been isolated and seq...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1007/BF01108407

    authors: Kaplan RS,Mayor JA

    更新日期:1993-10-01 00:00:00

  • Chemotherapy through mitochondrial apoptosis using nutritional supplements and herbs: a brief overview.

    abstract::There has been increased interest in the use of naturally occurring compounds with chemopreventive and chemotherapeutic effects in the treatment of cancers. This review summarizes the most recent advances that provide new insights into the molecular mechanisms underlying the apoptotic potential of nutritional suppleme...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章,评审

    doi:10.1007/s10863-006-9056-8

    authors: Kwon KB,Park BH,Ryu DG

    更新日期:2007-02-01 00:00:00

  • Function of FXYD proteins, regulators of Na, K-ATPase.

    abstract::In this short review, we summarize our work on the role of members of the FXYD protein family as tissue-specific modulators of Na, K-ATPase. FXYD1 or phospholemman, mainly expressed in heart and skeletal muscle increases the apparent affinity for intracellular Na(+) of Na, K-ATPase and may thus be important for approp...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章,评审

    doi:10.1007/s10863-005-9476-x

    authors: Geering K

    更新日期:2005-12-01 00:00:00

  • Respiratory and TCA cycle activities affect S. cerevisiae lifespan, response to caloric restriction and mtDNA stability.

    abstract::We studied the importance of respiratory fitness in S. cerevisiae lifespan, response to caloric restriction (CR) and mtDNA stability. Mutants harboring mtDNA instability and electron transport defects do not respond to CR, while tricarboxylic acid cycle mutants presented extended lifespans due to CR. Interestingly, mt...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1007/s10863-011-9377-0

    authors: Tahara EB,Cezário K,Souza-Pinto NC,Barros MH,Kowaltowski AJ

    更新日期:2011-10-01 00:00:00

  • Cytochrome c oxidase (heme aa3) from Paracoccus denitrificans: analysis of mutations in putative proton channels of subunit I.

    abstract::One of the challenging features of energy-transducing terminal oxidases, like the aa3 cytochrome c oxidase of Paracoccus denitrificans, is the translocation of protons across the cytoplasmic membrane, which is coupled to the transfer of electrons to oxygen. As a prerequisite for a more advanced examination of the enzy...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章,评审

    doi:10.1023/a:1020515713103

    authors: Pfitzner U,Odenwald A,Ostermann T,Weingard L,Ludwig B,Richter OM

    更新日期:1998-02-01 00:00:00

  • Energetics of isolated hepatocyte swelling induced by sodium co-transported amino acids.

    abstract::This study was designed to investigate the energetics of isolated rat hepatocyte swelling due to sodium-cotransported amino acid accumulation in a medium containing either glucose or octanoate as basal substrate. We show that the size of the increase in cytosolic volume is directly correlated with the total amino acid...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1023/a:1022487119390

    authors: Espié P,Devin A,Guérin B,Rigoulet M

    更新日期:1997-12-01 00:00:00

  • Drug-stimulated ATPase activity of the human P-glycoprotein.

    abstract::The human multidrug resistance protein, or P-glycoprotein (Pgp), exhibits a high-capacity drug-dependent ATP hydrolytic activity that is a direct reflection of its drug transport capability. This activity is readily measured in membranes isolated from cultured insect cells infected with a baculovirus carrying the huma...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章,评审

    doi:10.1007/BF02110329

    authors: Scarborough GA

    更新日期:1995-02-01 00:00:00

  • Mitochondrial matters of the brain: mitochondrial dysfunction and oxidative status in epilepsy.

    abstract::Epilepsy is a neurological disorder characterized by spontaneous, recurrent and paroxysmal cerebral discharge, clinically leading to persistent alterations in function and morphology of neurons. Oxidative stress is one of possible mechanisms in the pathogenesis of epilepsy. Oxidative stress resulting from mitochondria...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章,评审

    doi:10.1007/s10863-010-9317-4

    authors: Chang SJ,Yu BC

    更新日期:2010-12-01 00:00:00

  • Effects of methylglyoxal and pyridoxamine in rat brain mitochondria bioenergetics and oxidative status.

    abstract::Advanced glycation end products (AGEs) and methylglyoxal (MG), an important intermediate in AGEs synthesis, are thought to contribute to protein aging and to the pathogenesis of age-and diabetes-associated complications. This study was intended to investigate brain mitochondria bioenergetics and oxidative status of ra...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1007/s10863-014-9551-2

    authors: Cardoso S,Carvalho C,Marinho R,Simões A,Sena CM,Matafome P,Santos MS,Seiça RM,Moreira PI

    更新日期:2014-10-01 00:00:00

  • Molecular chaperones and mitochondrial protein folding.

    abstract::Precursor proteins destined for the mitochondrial matrix traverse inner and outer organelle membranes in an extended conformation. Translocation events are therefore integrally coupled to the processes of protein unfolding in the cytosol and protein refolding in the matrix. To successfully import proteins from the cyt...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1023/a:1022407705182

    authors: Martin J

    更新日期:1997-02-01 00:00:00

  • Parkinson's disease and mitochondrial complex I: a perspective on the Ndi1 therapy.

    abstract::Mitochondrial impairment has been collecting more and more attention as a contributing factor to the etiology of Parkinson's disease. Above all, the NADH-quinone oxidoreductase, complex I, of the respiratory chain seems to be most culpable. Complex I dysfunction is translated to an increased production of reactive oxy...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章,评审

    doi:10.1007/s10863-009-9249-z

    authors: Marella M,Seo BB,Yagi T,Matsuno-Yagi A

    更新日期:2009-12-01 00:00:00

  • Experimental production of "septa" and apparent subdivision of muscle mitochondria.

    abstract::The rapid formation (in less than 45 min) of internal septa and the apparent subdivision in situ of mitochondria for cardiac and skeletal muscle are described following a variety of experimental treatments. For example, the ionophore A23187, caffeine, DNP, ruthenium red, and the insecticide lindane have been applied t...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1007/BF00745010

    authors: Duncan CJ,Greenaway HC,Publicover SJ,Rudge MF,Smith JL

    更新日期:1980-04-01 00:00:00

  • Vacuolar H+ pumping ATPases in luminal acidic organelles and extracellular compartments: common rotational mechanism and diverse physiological roles.

    abstract::Cytoplasmic organelles with an acidic luminal pH include vacuoles, coated vesicles, lysosomes, the Golgi apparatus, and synaptic vesicles. Acidic compartments are also known outside specialized cells such as osteoclasts. The unique acidic pH is formed by V-ATPase (Vacuolar type ATPase), other ion transporters, and the...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章,评审

    doi:10.1023/a:1025780932403

    authors: Sun-Wada GH,Wada Y,Futai M

    更新日期:2003-08-01 00:00:00

  • Nonuniform lateral distribution of transmembrane deltapH in a coupling membrane as related to its curvature.

    abstract::The possibility is analyzed that the pH of the water space localized inside the invagination of a membrane can differ from the pH of the external bulk buffer outside the invagination. The proton flow responsible for decreased pH values inside mitochondrial cristae and membrane invaginations of cyanobacteria has been c...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1007/BF00745047

    authors: Kara-Ivanov M

    更新日期:1983-06-01 00:00:00

  • Morphology of proteoliposomes containing fluorescein-phosphatidylethanolamine reconstituted with native and subunit III-depleted cytochrome c oxidase.

    abstract::Beef heart cytochrome c oxidase was reconstituted in asolectin liposomes containing the pH indicator fluorescein-phosphatidylethanolamine (FPE) by the cholate-dialysis procedure. The influence of FPE on the asolectin liposome size and of the removal of subunit III from the complex on its incorporation into liposomes w...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1007/BF00743111

    authors: Müller M,Azzi A

    更新日期:1985-12-01 00:00:00

  • Osmomechanics of the Propionigenium modestum F(o) motor.

    abstract::In Propionigenium modestum, ATP is manufactured from ADP and phosphate by the enzyme ATP synthase using the free energy of an electrochemical gradient of Na+ ions. The P. modestum ATP synthase is a clear member of the family of F-type ATP synthases and the only major distinction is an extension of the coupling ion spe...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1023/a:1005608823087

    authors: Dimroth P,Matthey U,Kaim G

    更新日期:2000-10-01 00:00:00

  • Biological electron transfer.

    abstract::Many oxidoreductases are constructed from (a) local sites of strongly coupled substrate-redox cofactor partners participating in exchange of electron pairs, (b) electron pair/single electron transducing redox centers, and (c) nonadiabatic, long-distance, single-electron tunneling between weakly coupled redox centers. ...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章,评审

    doi:10.1007/BF02110096

    authors: Moser CC,Page CC,Farid R,Dutton PL

    更新日期:1995-06-01 00:00:00

  • The unraveling architecture of the junctional sarcoplasmic reticulum.

    abstract::The sarcoplasmic reticulum (SR) of skeletal muscle controls the contraction-relaxation cycle by raising and lowering the myoplasmic free-Ca2+ concentration. The coupling between excitation, i.e., depolarization of sarcolemma and transverse tubule (TT) and Ca2+ release from the terminal cisternae (TC) of SR takes place...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章,评审

    doi:10.1007/BF00812069

    authors: Volpe P

    更新日期:1989-04-01 00:00:00

  • Cross regulation between mTOR signaling and O-GlcNAcylation.

    abstract::The hexosamine biosynthetic pathway (HBP) integrates glucose, amino acids, fatty acids and nucleotides metabolisms for uridine diphosphate N-acetylglucosamine (UDP-GlcNAc) synthesis. UDP-GlcNAc is the nucleotide sugar donor for O-linked β-N-acetylglucosaminylation (O-GlcNAcylation) processes. O-GlcNAc transferase (OGT...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1007/s10863-018-9747-y

    authors: Very N,Steenackers A,Dubuquoy C,Vermuse J,Dubuquoy L,Lefebvre T,El Yazidi-Belkoura I

    更新日期:2018-06-01 00:00:00

  • Unimolecular study of the interaction between the outer membrane protein OmpF from E. coli and an analogue of the HP(2-20) antimicrobial peptide.

    abstract::Little is known on antimicrobial peptide permeation through outer membrane channels in gram-negative bacteria. Herein, we probed at a single-molecule level the interaction of two different peptides, magainin 2 and HPA3P with OmpF from E. coli. HPA3P is an analogue of the antimicrobial peptide HP(2-20) isolated from th...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1007/s10863-010-9273-z

    authors: Apetrei A,Asandei A,Park Y,Hahm KS,Winterhalter M,Luchian T

    更新日期:2010-04-01 00:00:00

  • Electron microscopic structural analysis of Photosystem I, Photosystem II, and the cytochrome b6/f complex from green plants and cyanobacteria.

    abstract::Electron microscopy (EM) in combination with image analysis is a powerful technique to study protein structure at low- and high resolution. Since electron micrographs of biological objects are very noisy, substantial improvement of image quality can be obtained by averaging individual projections. Crystallographic and...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章,评审

    doi:10.1007/BF00763217

    authors: Boekema EJ,Boonstra AF,Dekker JP,Rögner M

    更新日期:1994-02-01 00:00:00

  • Low-voltage-activated ("T-Type") calcium channels in review.

    abstract::The past 5 years has witnessed an advance in our understanding of alpha1G (Cav3.1), alpha1H (Cav3.2), and alpha11 (Car3.3), the pore-forming subunits of T-type or low-voltage-activated calcium channels (LVAs). LVAs differ in their localization and molecular, biophysical, and biochemical properties, but all conduct a t...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章,评审

    doi:10.1023/b:jobb.0000008024.77488.48

    authors: Yunker AM,McEnery MW

    更新日期:2003-12-01 00:00:00

  • Fatty acid synthesis by Chlamydomonas reinhardtii in phosphorus limitation.

    abstract::The importance of crucial nutrient factors like Phosphate (P) and their limited availability leads to variable fluctuations in fatty acid and phospholipid synthesis in the green alga. These fatty acids and phospholipids are an imperative byproduct of alga which used in biofuel production. The production of phospholipi...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1007/s10863-019-09813-8

    authors: Qari HA,Oves M

    更新日期:2020-02-01 00:00:00

  • Proton conductance through phospholipid bilayers: water wires or weak acids?

    abstract::The proton/hydroxide (H+/OH-) permeability of phospholipid bilayer membranes at neutral pH is at least five orders of magnitude higher than the alkali or halide ion permeability, but the mechanism(s) of H+/OH- transport are unknown. This review describes the characteristics of H+/OH- permeability and conductance throu...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章,评审

    doi:10.1007/BF00770028

    authors: Gutknecht J

    更新日期:1987-10-01 00:00:00

  • Redox-linked proton translocation by direct-coupled ligand conduction.

    abstract::The term "direct-coupled" is considered in the context of redox-linked proton translocation mechanisms, and the origins of this concept, its philosophical implications, applications, and contributions to the development of bioenergetics, are discussed. ...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章,评审

    doi:10.1007/BF00785997

    authors: West IC

    更新日期:1991-10-01 00:00:00

  • Purification of the channel component of the mitochondrial calcium uniporter and its reconstitution into planar lipid bilayers.

    abstract::The purification of the channel-forming component of the mitochondrial calcium uniporter and its channel properties are described. After ethanol and 50% ethanol-water extraction of mitochondria from beef heart or perfused rat liver, the extract was passed through thiopropyl-Sepharose 6B column, and absorbed components...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章

    doi:10.1007/BF00763072

    authors: Mironova GD,Baumann M,Kolomytkin O,Krasichkova Z,Berdimuratov A,Sirota T,Virtanen I,Saris NE

    更新日期:1994-04-01 00:00:00

  • Chemiosmotic concept of the membrane bioenergetics: what is already clear and what is still waiting for elucidation?

    abstract::The present state of the chemiosmotic concept is reviewed. Special attention is paid to (i) further progress in studies on the Na(+)-coupled energetics and (ii) paradoxical bioenergetic effects when protonic or sodium potentials are utilized outside the coupling membrane (TonB-mediated uphill transports across the out...

    journal_title:Journal of bioenergetics and biomembranes

    pub_type: 杂志文章,评审

    doi:10.1007/BF00831533

    authors: Skulachev VP

    更新日期:1994-12-01 00:00:00