Abstract：
:The ATP-binding cassette (ABC) family of membrane transport proteins is the largest class of transporters in humans (48 members). The majority of ABC transporters function at the cell surface. Therefore, defective folding and trafficking of the protein to the cell surface can lead to serious health problems. The classic example is cystic fibrosis (CF). In most CF patients, there is a deletion of Phe508 in the CFTR protein (DeltaF508 CFTR) that results in defective folding and intracellular retention of the protein (processing mutant). A potential treatment for most patients with CF would be to use a ligand(s) of CFTR that acts a pharmacological chaperone to correct the folding defect. The feasibility of such an approach was first demonstrated with the multidrug transporter P-glycoprotein (P-gp), an ABC transporter, and a sister protein of CFTR. It was found that P-gps with mutations at sites equivalent to those found in CFTR processing mutants were rescued when they were expressed in the presence of drug substrates or modulators of P-gp. These compounds acted as pharmacological chaperones and functioned by promoting interactions among the various domains in the protein during the folding process. Several groups have attempted to identify compounds that could rescue the folding defect in DeltaF508 CFTR. The best compound identified through high-throughout screening is a quinazoline derivative (CFcor-325). Expression of DeltaF508 CFTR as well as other CFTR processing mutants in the presence of 1 muM CFcor-325 promoted folding and trafficking of the mutant proteins to the cell surface in an active conformation. Therefore, CFcor-325 and other quinazoline derivates could be important therapeutic compounds for the treatment of CF.
journal_name
J Bioenerg Biomembrjournal_title
Journal of bioenergetics and biomembranesauthors
Loo TW,Bartlett MC,Clarke DMdoi
10.1007/s10863-005-9499-3subject
Has Abstractpub_date
2005-12-01 00:00:00pages
501-7issue
6eissn
0145-479Xissn
1573-6881journal_volume
37pub_type
杂志文章,评审abstract：:The respiratory chain of marine and moderately halophilic bacteria requires Na+ for maximum activity, and the site of Na(+)-dependent activation is located in the NADH-quinone reductase segment. The Na(+)-dependent NADH-quinone reductase purified from marine bacterium Vibrio alginolyticus is composed of three subunits...
journal_title：Journal of bioenergetics and biomembranes
pub_type： 杂志文章,评审
doi：10.1007/BF00762464
更新日期：1993-08-01 00:00:00
abstract：:Oxygen plays a key role in bacterial bioluminescence. The simultaneous and continuous kinetics of oxygen consumption and light emission during a complete exhaustion of the exogenous oxygen present in a closed system has been investigated. The kinetics are performed with Vibrio fischeri, V. harveyi, and Photobacterium ...
journal_title：Journal of bioenergetics and biomembranes
pub_type： 杂志文章
doi：10.1023/a:1010615508916
更新日期：2001-08-01 00:00:00
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
更新日期：2007-02-01 00:00:00
abstract：:High-conductance calcium-activated potassium (maxi-K) channels comprise a specialized family of K+ channels. They are unique in their dual requirement for depolarization and Ca2+ binding for transition to the open, or conducting, state. Ion conduction through maxi-K channels is blocked by a family of venom-derived pep...
journal_title：Journal of bioenergetics and biomembranes
pub_type： 杂志文章,评审
doi：10.1007/BF02110699
更新日期：1996-06-01 00:00:00
abstract：:The presence of transplasma membrane electron transport in a variety of plant cells and tissues is reported. It is now agreed that this property of eukaryotic cells is of ubiquitous nature. Studies with highly purified plasma membranes have established the presence of electron transport enzymes. Two types of activitie...
journal_title：Journal of bioenergetics and biomembranes
pub_type： 杂志文章,评审
doi：10.1007/BF00771013
更新日期：1991-06-01 00:00:00
abstract：:To discover the effect of tumor necrosis factor-alpha-induced protein 8-like 2 (TIPE2) on the oral tongue squamous cell carcinoma (OTSCC) via affecting FoxP3+ regulatory T (Treg) cells. Immunohistochemistry was conducted to examine TIPE2 and FoxP3 expressions in OTSCC tumor tissues and corresponding oral mucosa. Tca81...
journal_title：Journal of bioenergetics and biomembranes
pub_type： 杂志文章
doi：10.1007/s10863-020-09840-w
更新日期：2020-08-01 00:00:00
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
更新日期：2009-12-01 00:00:00
abstract：:Methylmalonic acidemia is one of the most prevalent inherited metabolic disorders involving neurological deficits. In vitro experiments, animal model studies and tissue analyses from human patients suggest extensive impairment of mitochondrial energy metabolism in this disease. This review summarizes changes in mitoch...
journal_title：Journal of bioenergetics and biomembranes
pub_type： 杂志文章,评审
doi：10.1007/s10863-011-9330-2
更新日期：2011-02-01 00:00:00
abstract：:Plasma membranes isolated from pig liver contained almost no acid sphingomyelinase but significant neutral magnesium-dependent sphingomyelinase that was activated by phosphatidylserine. We report here the purification to apparent homogeneity of neutral sphingomyelinase of about 87 kDa from liver plasma membranes. The ...
journal_title：Journal of bioenergetics and biomembranes
pub_type： 杂志文章
doi：10.1023/a:1010704715979
更新日期：2001-04-01 00:00:00
abstract：:The induction of millisecond delayed fluorescence mediated by PS I-dependent proton pumping has been used as an indicator of the time course with which those protons equilibrate with sites on the oxygen-evolving enzyme complex (Bowes, J. M., and Crofts, A. R. (1978). Z. Naturforsch. 33C, 271-275). We found that the in...
journal_title：Journal of bioenergetics and biomembranes
pub_type： 杂志文章
doi：10.1007/BF00769732
更新日期：1987-02-01 00:00:00
abstract：:Direct and indirect evidences show that elevated glucose concentrations in airway surface liquid (ASL) promote lung infection by pathogens, playing a role in the progression of the Cystic Fibrosis (CF) disease. The joint action of transporter/s for glucose and of the cellular enzymes is essential in order to try to lo...
journal_title：Journal of bioenergetics and biomembranes
pub_type： 杂志文章
doi：10.1007/s10863-019-09797-5
更新日期：2019-06-01 00:00:00
abstract：:Mitochondria, isolated from the ubiquinone-deficient nuclear mutant of Saccharomyces cerevisiae E3-24, are practically unable to oxidize exogenous substrates. Respiratory activity, coupled to ATP synthesis, can, however, be reconstituted by the simple addition of ethanolic solutions of ubiquinones. A minimal length of...
journal_title：Journal of bioenergetics and biomembranes
pub_type： 杂志文章
doi：10.1007/BF00745017
更新日期：1982-06-01 00:00:00
abstract：:Mitochondrial reactive oxygen species (ROS) production was investigated in mitochondria extracted from liver of rats treated with or without metformin, a mild inhibitor of respiratory chain complex 1 used in type 2 diabetes. A high rate of ROS production, fully suppressed by rotenone, was evidenced in non-phosphorylat...
journal_title：Journal of bioenergetics and biomembranes
pub_type： 杂志文章
doi：10.1007/s10863-006-9003-8
更新日期：2006-02-01 00:00:00
abstract：:Free radical production and leak of brain nonsynaptic mitochondria were higher with pyruvate/malate than with succinate in rats and pigeons. Rotenone, antimycin A, and myxothiazol maximally stimulated free radical production with pyruvate/malate but not with succinate. Simultaneous treatment with myxothiazol plus anti...
journal_title：Journal of bioenergetics and biomembranes
pub_type： 杂志文章
doi：10.1023/a:1020592719405
更新日期：1998-06-01 00:00:00
abstract：:Recent studies of isolated muscle membrane have enabled induction and monitoring of rapid Ca2+ release from sarcoplasmic reticulum (SR)5 in vitro by a variety of methods. On the other hand, various proteins that may be directly or indirectly involved in the Ca2+ release mechanism have begun to be unveiled. In this min...
journal_title：Journal of bioenergetics and biomembranes
pub_type： 杂志文章,评审
doi：10.1007/BF00812071
更新日期：1989-04-01 00:00:00
abstract：:Mitochondrial cytochrome c (horse), which is a very efficient electron donor to bacterial photosynthetic reaction centers in vitro, binds to the reaction center of Rhodospirillum rubrum with an approximate dissociation constant of 0.3-0.5 microM at pH 8.2 and low ionic strength. The binding site for the reaction cente...
journal_title：Journal of bioenergetics and biomembranes
pub_type： 杂志文章
doi：10.1007/BF00768540
更新日期：1987-08-01 00:00:00
abstract：:Cellular Ca2+ signals are crucial in the control of most physiological processes, cell injury and programmed cell death; mitochondria play a pivotal role in the regulation of such cytosolic Ca2+ ([Ca2+]c) signals. Mitochondria are endowed with multiple Ca2+ transport mechanisms by which they take up and release Ca2+ a...
journal_title：Journal of bioenergetics and biomembranes
pub_type： 杂志文章,评审
doi：10.1023/a:1005508311495
更新日期：2000-02-01 00:00:00
abstract：:Escherichia coli has four [NiFe]-hydrogenases (Hyd); three of these, Hyd-1, Hyd-2 and Hyd-3 have been characterized well. In this study the requirement for the F(0)F(1)-ATP synthase for the activities of the hydrogen-oxidizing hydrogenases Hyd-1 and Hyd-2 was examined. During fermentative growth on glucose at pH 7.5 a...
journal_title：Journal of bioenergetics and biomembranes
pub_type： 杂志文章
doi：10.1007/s10863-011-9397-9
更新日期：2011-12-01 00:00:00
abstract：:Mitochondria are dynamic structures for which fusion and fission are well characterized for rapidly dividing cells in culture. Based on these data, it has recently been proposed that high respiratory activity is the result of fusion and formation of mitochondrial reticulum, while fission results in fragmented mitochon...
journal_title：Journal of bioenergetics and biomembranes
pub_type： 杂志文章,评审
doi：10.1007/s10863-012-9494-4
更新日期：2013-08-01 00:00:00
abstract：:The development of new anti-neoplastic drugs is a key issue for cancer chemotherapy due to the reality that, most likely, certain cancer cells are resistant to current chemotherapy. The past two decades have witnessed tremendous advances in our understanding of the pathogenesis of cancer. These advances have allowed i...
journal_title：Journal of bioenergetics and biomembranes
pub_type： 杂志文章
doi：10.1007/s10863-012-9489-1
更新日期：2013-02-01 00:00:00
abstract：:The serotonergic system plays a crucial role in the energy balance regulation. Energy balance is mediated by food intake and caloric expenditure. Thus, the present study investigated the mechanisms that might be associated with fluoxetine treatment-induced weight reduction. Wistar male rat pups received daily injectio...
journal_title：Journal of bioenergetics and biomembranes
pub_type： 杂志文章
doi：10.1007/s10863-015-9617-9
更新日期：2015-08-01 00:00:00
abstract：:Although the "Warburg effect", i.e., elevated glucose metabolism to lactic acid (glycolysis) even in the presence of oxygen, has been recognized as the most common biochemical phenotype of cancer for over 80 years, its biochemical and genetic basis remained unknown for over 50 years. Work focused on elucidating the un...
journal_title：Journal of bioenergetics and biomembranes
pub_type： 社论
doi：10.1007/s10863-012-9425-4
更新日期：2012-02-01 00:00:00
abstract：:The original version of this article unfortunately contained a mistake. The name of "Zohreh Noaparast" is now corrected in the author group of this article. ...
journal_title：Journal of bioenergetics and biomembranes
pub_type： 已发布勘误
doi：10.1007/s10863-019-09803-w
更新日期：2019-08-01 00:00:00
abstract：:The mitochondrial inner membrane anion channel (IMAC) is a channel, identified by flux studies in intact mitochondria, which has a broad anion selectivity and is maintained closed or inactive by matrix Mg2+ and H+. We now present evidence that this channel, like many other chloride/anion channels, is reversibly blocke...
journal_title：Journal of bioenergetics and biomembranes
pub_type： 杂志文章
doi：10.1007/BF02110652
更新日期：1996-04-01 00:00:00
abstract：:The bacterial phosphoenolpyruvate-dependent carbohydrate phosphotransferase system (PTS) consists of several proteins whose primary functions are to transport and phosphorylate their substrates. The complexity of the PTS undoubtedly reflects its additional roles in chemotaxis to PTS substrates and in regulation of oth...
journal_title：Journal of bioenergetics and biomembranes
pub_type： 杂志文章,评审
doi：10.1007/BF00770249
更新日期：1993-12-01 00:00:00
abstract：:Absorbance changes at 446 nm in purified cytochrome oxidase following flash photolysis of carboxy-oxidase poised in the mixed valance state at +220 mV show biphasic kinetics. One phase corresponds to CO recombination to ferrous cytochrome a3 with an energy of activation of 9 kcal/mol; the second phase is 3-5 times fas...
journal_title：Journal of bioenergetics and biomembranes
pub_type： 杂志文章
doi：10.1007/BF00762550
更新日期：1988-12-01 00:00:00
abstract：:We have studied the effect of general anesthetics on the mobility of two stearic acid spin labels (5-doxyl stearic acid and 16-doxyl stearic acid) in bovine heart mitochondria and in phospholipid vesicles made from either mitochondrial lipids or commercial soybean phospholipids. The general anesthetics used include no...
journal_title：Journal of bioenergetics and biomembranes
pub_type： 杂志文章
doi：10.1007/BF00743158
更新日期：1979-04-01 00:00:00
abstract：:P-glycoprotein (Pgp), the ATP-binding cassette (ABC) transporter, confers multidrug resistance to cancer cells by extruding cytotoxic natural product amphipathic drugs using the energy of ATP hydrolysis. Our studies are directed toward understanding the mechanism of action of Pgp and recent work deals with the assessm...
journal_title：Journal of bioenergetics and biomembranes
pub_type： 杂志文章,评审
doi：10.1023/a:1012875105006
更新日期：2001-12-01 00:00:00
abstract：:Caloric restriction (CR) is widely known to increase life span and resistance to different types of injuries in several organisms. We have previously shown that mitochondria from livers or brains of CR animals exhibit higher calcium uptake rates and lower sensitivity to calcium-induced mitochondrial permeability trans...
journal_title：Journal of bioenergetics and biomembranes
pub_type： 杂志文章
doi：10.1007/s10863-020-09838-4
更新日期：2020-08-01 00:00:00
abstract：:The organization of the oxidative phosphorylation (OXPHOS) system within the inner mitochondrial membrane appears to be far more complicated than previously thought. In particular, the individual protein complexes of the OXPHOS system (complexes I to V) were found to specifically interact forming defined supramolecula...
journal_title：Journal of bioenergetics and biomembranes
pub_type： 杂志文章,评审
doi：10.1007/s10863-008-9167-5
更新日期：2008-10-01 00:00:00
