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Página 1 desde 16 resultados
Lax eyelid syndrome or 'progeria' of eyelid tissues.
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The lax eyelid syndrome was described by Van den Bosch and Lemij as an uncommon disorder seen in non-obese elderly people and characterised by chronic ocular surface irritation symptoms and a "floppy upper eyelid". The authors present some new features of the lax eyelid
Leg ulcer in Werner syndrome (adult progeria): a case report.
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Werner syndrome (WS; MIM#277700) or adult progeria, is a rare disease, associated with mutations of a single gene (RECQL2 or WRN), located on chromosome 8 (8p12). It codes a DNA-helicase, whose defects cause genomic instability. The highest incidences are reported in Japan and Sardinia (Italy). On
Progeria, rapamycin and normal aging: recent breakthrough.
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A recent discovery that rapamycin suppresses a pro-senescent phenotype in progeric cells not only suggests a non-toxic therapy for progeria but also implies its similarity with normal aging. For one, rapamycin is also known to suppress aging of regular human cells. Here I discuss four potential
Are There Common Mechanisms Between the Hutchinson-Gilford Progeria Syndrome and Natural Aging?
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The Hutchinson-Gilford progeria syndrome (HGPS) is a premature aging disease caused by mutations of the LMNA gene leading to increased production of a partially processed form of the nuclear fibrillar protein lamin A - progerin. Progerin acts as a dominant factor that leads to multiple
Schizophrenia as segmental progeria.
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Schizophrenia is associated with a variety of physical manifestations (i.e. metabolic, neurological) and despite psychotropic medication being blamed for some of these (in particular obesity and diabetes), there is evidence that schizophrenia itself confers an increased risk of physical disease and
Effect of Tokaj wine in combination with water and 10% alcohol solution on long-life and development of alcohol progeria in Wistar SPF rats.
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Whole-life consumption of the pure Tokaj wine (The Furmint variety) with documented antioxidative activity significantly elongated (p<0.05) the life of laboratory animals in comparison to the control group consuming water and in comparison to group consuming ethanol (10%). Whole-life intake of
Assessment of the Aging of the Brown Adipose Tissue by 18F-FDG PET/CT Imaging in the Progeria Mouse Model Lmna-/.
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Brown adipose tissue (BAT) is an important energy metabolic organ that is highly implicated in obesity, type 2 diabetes, and atherosclerosis. Aging is one of the most important determinants of BAT activity. In this study, we used 18F-FDG PET/CT imaging to assess BAT aging in Lmna-/- mice. The
Epigenomic maintenance through dietary intervention can facilitate DNA repair process to slow down the progress of premature aging.
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DNA damage caused by various sources remains one of the most researched topics in the area of aging and neurodegeneration. Increased DNA damage causes premature aging. Aging is plastic and is characterised by the decline in the ability of a cell/organism to maintain genomic stability. Lifespan can
ZMPSTE24 Is Associated with Elevated Inflammation and Progerin mRNA
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Lamins are important filaments forming the inner nuclear membrane. Lamin A is processed by zinc metalloproteinase (ZMPSTE24). Failure to cleave a truncated form of prelamin A-also called progerin-causes Hutchinson-Gilford progeria syndrome a well-known premature aging disease. Minor levels of
Evaluation of glucose tolerance and effect of dietary management on increased visceral fat in a patient with Werner syndrome
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Werner syndrome (WS), a type of progeria, is a hereditary condition caused by a mutation in the WRN gene. A 62-year-old Japanese woman was diagnosed with WS at the age of 32 and has been visiting the hospital for follow-up since the last 30 years. The patient developed diabetes at the age of 46, and
Monogenic forms of insulin resistance: apertures that expose the common metabolic syndrome.
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Insulin resistance is common and plays a central role in the pathogenesis of type 2 diabetes mellitus (T2DM). Precedents in biomedical research indicate that evaluation of monogenic syndromes can help to understand a common complex phenotype. Monogenic forms of insulin resistance, such as familial
Adipokines and aging.
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Dysregulation of adipose tissue-derived bioactive molecules, termed adipokines, is recognized as common ground for insulin resistance and metabolic syndrome associated with obesity. However, adipokine dysregulation is paradoxically associated with lipodystrophy and lipoatrophy with aging. In
Growing Old Too Fast: A Rare Case of Werner Syndrome.
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Werner syndrome (WS) is rare adult-onset progeria characterized by premature aging and early death. Patients develop normally until adolescence and usually present in early adulthood. Our case highlights a common presentation of this uncommon disease, wherein a 29-year-old non-obese male with no
A Heterozygous ZMPSTE24 Mutation Associated with Severe Metabolic Syndrome, Ectopic Fat Accumulation, and Dilated Cardiomyopathy.
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ZMPSTE24 encodes the only metalloprotease, which transforms prelamin into mature lamin A. Up to now, mutations in ZMPSTE24 have been linked to Restrictive Dermopathy (RD), Progeria or Mandibulo-Acral Dysplasia (MAD). We report here the phenotype of a patient referred for severe metabolic syndrome
Dicer1-miR-328-Bace1 signalling controls brown adipose tissue differentiation and function.
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Activation of brown adipose tissue (BAT) controls energy homeostasis in rodents and humans and has emerged as an innovative strategy for the treatment of obesity and type 2 diabetes mellitus. Here we show that ageing- and obesity-associated dysfunction of brown fat coincides with global microRNA
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