Articles with "hutchinson gilford" as a keyword



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Microbiome at sites of gingival recession in children with Hutchinson–Gilford progeria syndrome

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Published in 2018 at "Journal of Periodontology"

DOI: 10.1002/jper.17-0351

Abstract: BACKGROUND Hutchinson-Gilford progeria syndrome (HGPS) is a rare premature aging disorder with significant oral and dental abnormalities. Clinical symptoms include various features of accelerated aging such as alopecia, loss of subcutaneous fat, bone abnormalities, and… read more here.

Keywords: hutchinson gilford; gingival recession; sites gingival; progeria syndrome ... See more keywords
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Pharmacotherapy to gene editing: potential therapeutic approaches for Hutchinson–Gilford progeria syndrome

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Published in 2020 at "GeroScience"

DOI: 10.1007/s11357-020-00167-3

Abstract: Hutchinson–Gilford progeria syndrome (HGPS), commonly called progeria, is an extremely rare disorder that affects only one child per four million births. It is characterized by accelerated aging in affected individuals leading to premature death at… read more here.

Keywords: hgps; hutchinson gilford; disorder; therapeutic approaches ... See more keywords
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Cardiovascular manifestations of Hutchinson-Gilford progeria syndrome.

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Published in 2022 at "Cardiology in the young"

DOI: 10.1017/s104795112200004x

Abstract: Cardiovascular complications are the most frequent cause of death in patients with the Hutchinson-Gilford progeria syndrome. However, due to its rarity, studying the course of cardiac abnormalities has been a challenge. The cardiovascular phenotype helps… read more here.

Keywords: cardiovascular manifestations; progeria syndrome; gilford progeria; hutchinson gilford ... See more keywords
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The clinical characteristics of Asian patients with classical-type Hutchinson–Gilford progeria syndrome

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Published in 2017 at "Journal of Human Genetics"

DOI: 10.1038/jhg.2017.90

Abstract: Hutchinson–Gilford progeria syndrome (HGPS) is an extremely rare genetic disorder that shows a characteristic progeria phenotype. We conducted a questionnaire survey of 1173 tertiary hospitals in Japan and reviewed the academic reports, to identify the… read more here.

Keywords: hutchinson gilford; characteristics asian; asian patients; progeria syndrome ... See more keywords
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An overview of treatment strategies for Hutchinson-Gilford Progeria syndrome

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Published in 2018 at "Nucleus"

DOI: 10.1080/19491034.2018.1460045

Abstract: ABSTRACT Hutchinson-Gilford progeria syndrome (HGPS) is a sporadic, autosomal dominant disorder characterized by premature and accelerated aging symptoms leading to death at the mean age of 14.6 years usually due to cardiovascular complications. HGPS is… read more here.

Keywords: hutchinson gilford; treatment strategies; overview treatment; progeria syndrome ... See more keywords
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Transcriptional profiling of Hutchinson-Gilford Progeria syndrome fibroblasts reveals deficits in mesenchymal stem cell commitment to differentiation related to early events in endochondral ossification

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Published in 2022 at "eLife"

DOI: 10.1101/2022.06.21.497024

Abstract: Hutchinson-Gilford Progeria Syndrome results from a mutation in Lamin A, and it is characterized by the incorporation of progerin into the nuclear lamina. Progerin expression leads to alterations in genome architecture, nuclear morphology, and epigenetic… read more here.

Keywords: endochondral ossification; progeria syndrome; gilford progeria; hutchinson gilford ... See more keywords
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Lysophosphatidic acid receptor LPA3 prevents oxidative stress and cellular senescence in Hutchinson–Gilford progeria syndrome

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Published in 2019 at "Aging Cell"

DOI: 10.1111/acel.13064

Abstract: Hutchinson–Gilford progeria syndrome (HGPS) is a rare laminopathy that produces a mutant form of prelamin A, known as Progerin, resulting in premature aging. HGPS cells show morphological abnormalities of the nuclear membrane, reduced cell proliferation… read more here.

Keywords: hutchinson gilford; senescence; progeria syndrome; lysophosphatidic acid ... See more keywords
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DNA methylation signatures in Blood DNA of Hutchinson–Gilford Progeria syndrome

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Published in 2022 at "Aging Cell"

DOI: 10.1111/acel.13555

Abstract: Hutchinson–Gilford Progeria Syndrome (HGPS) is an extremely rare genetic disorder caused by mutations in the LMNA gene and characterized by premature and accelerated aging beginning in childhood. In this study, we performed the first genome‐wide… read more here.

Keywords: methylation; dna; gilford progeria; hutchinson gilford ... See more keywords
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Plasma Progerin in Patients With Hutchinson-Gilford Progeria Syndrome: Immunoassay Development and Clinical Evaluation

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Published in 2023 at "Circulation"

DOI: 10.1161/circulationaha.122.060002

Abstract: Background: Hutchinson-Gilford progeria syndrome (HGPS) is an ultrarare, fatal, premature aging disease caused by a toxic protein called progerin. Circulating progerin has not been previously detected, precluding research using readily available biological samples. This study… read more here.

Keywords: decrease; progerin; gilford progeria; plasma progerin ... See more keywords
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Next-Generation Sequencing and Quantitative Proteomics of Hutchinson-Gilford progeria syndrome-derived cells point to a role of nucleotide metabolism in premature aging

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Published in 2018 at "PLoS ONE"

DOI: 10.1371/journal.pone.0205878

Abstract: Hutchinson-Gilford progeria syndrome (HGPS) is a very rare fatal disease characterized for accelerated aging. Although the causal agent, a point mutation in LMNA gene, was identified more than a decade ago, the molecular mechanisms underlying… read more here.

Keywords: hutchinson gilford; cell lines; progeria syndrome; gilford progeria ... See more keywords
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Hutchinson-Gilford Progeria Syndrome: An Overview of the Molecular Mechanism, Pathophysiology and Therapeutic Approach.

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Published in 2021 at "Current gene therapy"

DOI: 10.2174/1566523221666210303100805

Abstract: Lamin A/C encoded by LMNA gene is an important component for the maintenance of the nuclear structure. Mutation in the lamin A/C leads to a group of inherited disorders is known as laminopathies. In the… read more here.

Keywords: hutchinson gilford; progeria syndrome; molecular mechanism; gilford progeria ... See more keywords