Abstract：
:Mutated huntingtin is expressed in nervous and non nervous system included lymphoblasts. Eneregetic metabolism is impaired in Huntington's disease (HD) and other neurodegenerative diseases. Human HD lymphoblasts have provided clear-cut data on mitochondnal disruption. Here we report morphological, morphometric and membrane potential differences in mitochondria from lymphoblasts obtained from patients homozygous and heterozygous for the CAG mutation, and controls. Homozygotes, who despite a similar age at onset show a more aggressive phenotype than heterozygotes, had giant mitochondria and a reduced membrane potential. We argue that early mitochondrial impairment at basal level may affect the severity of HD progression in patients.
journal_name
Mech Ageing Devjournal_title
Mechanisms of ageing and developmentauthors
Squitieri F,Cannella M,Sgarbi G,Maglione V,Falleni A,Lenzi P,Baracca A,Cislaghi G,Saft C,Ragona G,Russo MA,Thompson LM,Solaini G,Fornai Fdoi
10.1016/j.mad.2005.09.010subject
Has Abstractpub_date
2006-02-01 00:00:00pages
217-20issue
2eissn
0047-6374issn
1872-6216pii
S0047-6374(05)00228-9journal_volume
127pub_type
杂志文章abstract：:Age related changes in some aspects of glutamate metabolism were followed in the brain of a short lived species of tropical murrel, Channa punctatus. Both ammonia and glutamine contents of brain increased with advancing age of the murrel. The glutamate dehydrogenase activity of brain increased during maturation phase ...
journal_title：Mechanisms of ageing and development
pub_type： 杂志文章
doi：10.1016/0047-6374(93)90139-i
更新日期：1993-05-01 00:00:00
abstract：:The age-related changes in the distribution of enkephalin-immunoreactive (ENK-IR) nerve fibers in relation to the principal neurons were studied in 23 human sympathetic ganglia from patients aged between 22 and 98 years. There were no age-related changes in the diameter of nerve cells and the packing density of the ne...
journal_title：Mechanisms of ageing and development
pub_type： 杂志文章
doi：10.1016/0047-6374(88)90086-3
更新日期：1988-08-01 00:00:00
abstract：:The purpose of this research is to determine possible causes and mechanisms involved in the age-associated decline in mitochondrial activity. We have focused on cytochrome c oxidase because it is comprised of both nuclear and mitochondrial-encoded subunits and may provide some insight into the coordination of the two ...
journal_title：Mechanisms of ageing and development
pub_type： 杂志文章
doi：10.1016/s0047-6374(98)00043-8
更新日期：1998-06-15 00:00:00
abstract：:As measured by X-ray microanalysis of frozen-dried cryosections, physiological concentrations of phosphorus, Na+, K+ and Mg2+ show a biphasic age dependence in some compartments of rat hepatocytes, especially in the regions of condensed chromatin associated with the actively transcribed genes. This could be consistent...
journal_title：Mechanisms of ageing and development
pub_type： 杂志文章
doi：10.1016/0047-6374(87)90077-7
更新日期：1987-04-01 00:00:00
abstract：:Relationships between nutrition and longevity are of growing interest. Here we analysed the influences of dietary restriction on the survival of Drosophila exposed to atmospheric oxygen or to chronic hypoxia. Dietary restriction was achieved by food dilution, by sucrose restriction or by yeast restriction. Sucrose and...
journal_title：Mechanisms of ageing and development
pub_type： 杂志文章
doi：10.1016/j.mad.2007.05.008
更新日期：2007-05-01 00:00:00
abstract：:Hepatic growth and protein synthesis in vivo was studied with age in ad libitum-fed and dietary restricted rats in which the mean and maximum lifespan was significantly extended. Livers from underfed rats showed significantly lower DNA, RNA and protein contents, and total protein synthesis. The fractional rate of synt...
journal_title：Mechanisms of ageing and development
pub_type： 杂志文章
doi：10.1016/0047-6374(87)90008-x
更新日期：1987-07-01 00:00:00
abstract：:Chondrocytes derived from chick embryos can be successfully implanted in defects of adult chick articular cartilage surfaces. Such implants thrive in their implantation site and create a new articular surface. The chondrocytes mature and hypertrophy in the orthotopic site without invoking an immune response. Eventuall...
journal_title：Mechanisms of ageing and development
pub_type： 杂志文章
doi：10.1016/0047-6374(89)90060-2
更新日期：1989-10-01 00:00:00
abstract：INTRODUCTION:Our objective was to study the impact of an identical additional stress on male and female mortality with a quasi-experimental study design, using natural variations in summer mortality, including the massive heat wave that struck Europe in 2003. MATERIAL AND METHODS:The summer daily mortality rates of th...
journal_title：Mechanisms of ageing and development
pub_type： 杂志文章
doi：10.1016/j.mad.2012.04.004
更新日期：2012-06-01 00:00:00
abstract：:We have previously reported that two or more different subpopulations of bone marrow stem cells exist in mice as determined by cycling status of day-8 and day-14 CFU-S in long term bromodeoxyuridine (BrdU) infusion studies. In the present report, comparisons between cycling of stem cell subpopulations in old and young...
journal_title：Mechanisms of ageing and development
pub_type： 杂志文章
doi：10.1016/0047-6374(89)90069-9
更新日期：1989-07-01 00:00:00
abstract：:Caloric restriction has been extensively investigated as an intervention that both extends lifespan and delays age-related disease in mammals. In mice, much interest has centered on evaluating gene expression changes induced by caloric restriction (CR) in particular tissue types, but the overall systemic effect of CR ...
journal_title：Mechanisms of ageing and development
pub_type： 杂志文章
doi：10.1016/j.mad.2007.11.003
更新日期：2008-03-01 00:00:00
abstract：:The present study examined the effect of a 40% reduction in caloric intake (CR) versus ad libitum (AL) feeding on retinal aging. CR- and AL-fed Brown Norway (BN) rats were obtained at 12, 24 and 30 months of age from the National Center for Toxicological Research (NCTR). Age-dependent declines in outer nuclear layer (...
journal_title：Mechanisms of ageing and development
pub_type： 杂志文章
doi：10.1016/s0047-6374(00)00096-8
更新日期：2000-03-13 00:00:00
abstract：:Certain anatomic and functional parameters of the eye change with increasing chronological age. They may, therefore, serve as potential biomarkers of ageing. We investigated associations between four such ocular parameters (lens density, retinal vessel calibre, corneal endothelial cells and retinal nerve fibre layer t...
journal_title：Mechanisms of ageing and development
pub_type： 临床试验,杂志文章,多中心研究
doi：10.1016/j.mad.2013.05.002
更新日期：2013-07-01 00:00:00
abstract：:Cell proliferation, cell injury and aging are connected with changes in the cell membrane structure. Apparently these changes activate, in mammalian as well as in plant cells, lipases which liberate polyunsaturated fatty acids (PUFAs). PUFAs are the substrates for lipoxygenases which convert them to corresponding hydr...
journal_title：Mechanisms of ageing and development
pub_type： 杂志文章,评审
doi：10.1016/s0047-6374(01)00220-2
更新日期：2001-05-31 00:00:00
abstract：:Glucose phosphorylating activity of human erythrocytes quickly decreases during cell ageing; the electrophoretic pattern suggests that this fast decrease is due mainly to the isozyme II. We have shown that in the young cells only hexokinase I and II are responsible for the glucose phosphorylation, while in the old cel...
journal_title：Mechanisms of ageing and development
pub_type： 杂志文章
doi：10.1016/0047-6374(78)90024-6
更新日期：1978-10-01 00:00:00
abstract：:Possible alterations in the population of poly(A)(+)mRNA during ageing were investigated by translation in vitro of poly(A)(+)mRNA from the liver, kidney and brain of male ddY mice of different ages. [35S]Methionine-labeled translation products were analysed by two-dimensional polyacrylamide gel electrophoresis follow...
journal_title：Mechanisms of ageing and development
pub_type： 杂志文章
doi：10.1016/0047-6374(83)90063-5
更新日期：1983-10-01 00:00:00
abstract：:Scavenger receptor B1 (SR-B1) is a trans-membrane protein, involved in tissue reverse cholesterol transport. Several studies have demonstrated that SR-B1 is also implicated in other physiological processes, such as bacteria and apoptotic cells recognition and regulation of intracellular tocopherol and carotenoids leve...
journal_title：Mechanisms of ageing and development
pub_type： 杂志文章
doi：10.1016/j.mad.2017.11.006
更新日期：2018-06-01 00:00:00
abstract：:The strychnine-insensitive binding of [3H]glycine was characterized in purified cerebral cortical membranes from mice aged from 7 days to 22 months. The binding was saturable, exhibiting only one component during the whole life-span studied. The binding constant KD did not change during development and ageing, whereas...
journal_title：Mechanisms of ageing and development
pub_type： 杂志文章
doi：10.1016/0047-6374(93)90131-a
更新日期：1993-11-01 00:00:00
abstract：:Mitochondrial DNA (mtDNA) was isolated from liver mitochondria of rats between 2 and 24 months of age. The mtDNA was purified by cesium chloride--ethidium bromide isopycnic density gradient centrifugation. In the gradients, in addition to the two expected bands of ethidium--DNA complex, there was observed a third, mor...
journal_title：Mechanisms of ageing and development
pub_type： 杂志文章
doi：10.1016/0047-6374(82)90090-2
更新日期：1982-11-01 00:00:00
abstract：:The kinetics of inactivation of plasma albumin was studied in young (3-4 months) and old (25-28 months) Sprague-Dawley female rats. Conscious, free-moving animals carrying indwelling atrial and carotid cannulas received a single injection of [125I]-albumin (rat) via the carotid cannula. Sequential blood samples were r...
journal_title：Mechanisms of ageing and development
pub_type： 杂志文章
doi：10.1016/0047-6374(86)90119-3
更新日期：1986-11-28 00:00:00
abstract：:The bone marrow of old adult mice ( approximately 2 years old) has reduced B lymphopoiesis; however, whether the B1 pathway in adult bone marrow is also compromised in senescence is not known. Herein, we show that phenotypic (IgM(-)Lin(-)CD93(+)[AA4.1(+)] CD19(+)B220(low/-)) B1 progenitors are retained in old bone mar...
journal_title：Mechanisms of ageing and development
pub_type： 杂志文章
doi：10.1016/j.mad.2009.04.001
更新日期：2009-06-01 00:00:00
abstract：:Susceptibility for Friend leukemia virus (FLV)-induced leukemogenesis was examined in the C3H/He (C3H)-->C57BL/6 (B6) radiation bone marrow chimeras of various age groups, and the effect of aging of host mice on the susceptibility was determined. The bone marrow chimera system provided the various age of FLV-resistant...
journal_title：Mechanisms of ageing and development
pub_type： 杂志文章
doi：10.1016/s0047-6374(00)00245-1
更新日期：2001-02-01 00:00:00
abstract：:Filopodia in log and stationary phase populations of human fetal lung fibroblasts (WI-38) at low and high population doubling levels (PDLs) and of SV40 transformed WI-38 cells (VA13A), were observed and counted under different conditions of in vitro growth by scanning electron microscopy. Cells from old non-vigorously...
journal_title：Mechanisms of ageing and development
pub_type： 杂志文章
doi：10.1016/0047-6374(86)90048-5
更新日期：1986-02-01 00:00:00
abstract：:Epigenetic modifications, including changes in DNA methylation, have been implicated in a wide range of diseases including neurological diseases such as Alzheimer's. The role of dietary folate in providing methyl groups required for maintenance and modulation of DNA methylation makes it a nutrient of interest in Alzhe...
journal_title：Mechanisms of ageing and development
pub_type： 杂志文章,评审
doi：10.1016/j.mad.2017.10.001
更新日期：2018-09-01 00:00:00
abstract：:Compelling evidence supports the importance of metabolic abnormalities in Alzheimer disease pathogenesis. Indeed, that oxidative mechanisms are involved in the neuropathology associated with Alzheimer disease is evidenced by the large number of metabolic signs of oxidative stress as well as by specific markers of oxid...
journal_title：Mechanisms of ageing and development
pub_type： 杂志文章,评审
doi：10.1016/j.mad.2006.01.024
更新日期：2006-06-01 00:00:00
abstract：:The human premature aging protein Werner (WRN), deficient in Werner syndrome (WS), is localized mainly to the nucleolus in many cell types. DNA damage or replication arrest causes WRN to redistribute from the nucleolus to the nucleoplasm into discrete foci. In this study, we have investigated DNA damage specific cellu...
journal_title：Mechanisms of ageing and development
pub_type： 杂志文章
doi：10.1016/j.mad.2005.06.004
更新日期：2005-11-01 00:00:00
abstract：:Epidermal mass of the skin of Balb/c female mice of 2, 6, 12, and 20 months of age was determined by measuring epidermal wet weight, RNA, DNA, and protein per unit area (100 cm2) of skin. Epidermal wet weight is significantly decreased in 20-month-old mice compared to 2-month-old mice. This represents a real decrease ...
journal_title：Mechanisms of ageing and development
pub_type： 杂志文章
doi：10.1016/0047-6374(83)90088-x
更新日期：1983-07-01 00:00:00
abstract：:Neurons that degenerate in the brains of persons with Alzheimer's disease accumulate mitochondrial amyloid precursor protein (APP), which is thought to negatively affect mitochondrial function and cellular homeostasis. Because proteins that enter mitochondria require assistance from chaperone proteins, we hypothesized...
journal_title：Mechanisms of ageing and development
pub_type： 杂志文章
doi：10.1016/j.mad.2009.07.002
更新日期：2009-09-01 00:00:00
abstract：:This study aimed to assess platelets as a possible model for screening the accumulation of mitochondrial DNA mutations, particularly during normal ageing. For this purpose we isolated platelets from young and old donors selected by lack of systemic and haematological diseases. We studied the accumulation of a particul...
journal_title：Mechanisms of ageing and development
pub_type： 杂志文章
doi：10.1016/s0047-6374(97)00181-4
更新日期：1998-04-01 00:00:00
abstract：:The efficient use of nutrients is important in development and aging. In this study, we asked if the protein repair methyltransferase has a related or additional role in energy metabolism and stress response in the nematode Caenorhabditis elegans. Worms lacking the pcm-1 gene encoding this enzyme exhibit reduced longe...
journal_title：Mechanisms of ageing and development
pub_type： 杂志文章
doi：10.1016/j.mad.2008.09.019
更新日期：2008-12-01 00:00:00
abstract：:Rats of two age groups (6 weeks and 30 months) received (1) a single dose of 600 mg D-galactosamine (GalN)/kg body weight by intraperitoneal (i.p.) injection, (2) a single dose of 600 mg GalN/kg body weight i.p. combined with 20 mg prednisolone/kg body weight subcutaneously at the beginning of the experiment. The kine...
journal_title：Mechanisms of ageing and development
pub_type： 杂志文章
doi：10.1016/0047-6374(78)90064-7
更新日期：1978-03-01 00:00:00
