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Showing 1 to 20 of 67 for “"telangiectasia"”.

  1. Genetic Analysis of Macular Telangiectasia

    Macular telangiectasia type 2, or MacTel, is an adult onset retinal disease that causes progressive loss of central vision, usually beginning between the 5th and 7th decades of life. Macular degenerative diseases comprise a large portion of the blinding diseases affecting people over the age of 50. …

    columbia-diss Repository record for Genetic Analysis of Macular Telangiectasia (opens in a new tab)

  2. Exploring neurodegeneration in Ataxia-Telangiectasia

    Ataxia-Telangiectasia (A-T) is a very rare autosomal recessive DNA repair disorder. The condition is characterised by a progressive neurodegenerative disorder. Cancer predisposition, immunodeficiency and respiratory disease can result in premature death. The life expectancy of a patient with A-T is …

    cambridge Repository record for Exploring neurodegeneration in Ataxia-Telangiectasia (opens in a new tab)

  3. Validierung der Zellzyklusdiagnostik bei Ataxia telangiectasia

    … mit der klinischen Verdachtsdiagnose Ataxia telangiectasia beschrieben. Hierzu wurden die Daten von 327 Patienten ausgewertet. In 82 Fällen ergab sich eine Bestätigung der Verdachtsdiagnose, in 225 Fällen konnte das Vorliegen dieser Erkrankung ausgeschlossen werden, bei den übrigen …

    wurz-thes Repository record for Validierung der Zellzyklusdiagnostik bei Ataxia telangiectasia (opens in a new tab)

  4. How is Ataxia-Telangiectasia Mutated Protein Kinase Activated?

    Ataxia-Telangiectasia Mutated (ATM) is a key protein kinase in the cell’s response to double-stranded breaks in DNA. This damage is detected by the Mre11-Rad50-Nbs1 (MRN) complex, which recruits ATM to the DNA. Upon activation, ATM phosphorylates a vast range of substrates, which triggers a …

    cambridge Repository record for How is Ataxia-Telangiectasia Mutated Protein Kinase Activated? (opens in a new tab)

  5. The role of neuron-microglial interactions in Ataxia-Telangiectasia

    Ataxia-Telangiectasia (A-T) is a genome instability disorder characterised by progressive loss of cerebellar neurons, as well as metabolic and immunological deficits. A-T is caused by mutations in ATM kinase, a critical regulator of cellular response to DNA damage, oxidative stress, and more …

    cambridge Repository record for The role of neuron-microglial interactions in Ataxia-Telangiectasia (opens in a new tab)

  6. Mechanisms of cytosolic DNA sensing and neuroinflammation in ataxia-telangiectasia

    … in the central nervous system (CNS). Ataxia-telangiectasia (A-T) is a prototypical genome instability syndrome caused by loss-of-function mutations in ATM kinase, a master regulator of the cellular response to DNA damage, including DNA double stand breaks and oxidative stress. A-T is a …

    cambridge Repository record for Mechanisms of cytosolic DNA sensing and neuroinflammation in ataxia-telangiectasia (opens in a new tab)

  7. In vivo and in vitro studies of immunodeficiency in Ataxia-telangiectasia

    Ataxia-telangiectasia (A-T) is a rare neurodegenerative disorder caused by mutations in the ATM gene which has a central role in the cellular response to DNA double strand breaks, cell cycle checkpoint control and initiation of the intrinsic pathway of apoptosis. Ataxiatelangiectasia is classified …

    birmingham Repository record for In vivo and in vitro studies of immunodeficiency in Ataxia-telangiectasia (opens in a new tab)

  8. THE FUNCTION OF MRN (MRE11-RAD50-NBS1) COMPLEX DURING WRN (WERNER) FACILITATED ATM (ATAXIA-TELANGIECTASIA MUTATED) ACTIVATION

    … and premature aging disease. ATM (Ataxia-Telangiectasia mutated) protein initiates a signaling pathway in response to DNA double strand breaks (DSBs). Genomic disorder ataxia-telangiectasia (A-T) is associated with defective ATM. WRN protein is involved in ATM pathway activation when cells …

    maryland Repository record for THE FUNCTION OF MRN (MRE11-RAD50-NBS1) COMPLEX DURING WRN (WERNER) FACILITATED ATM (ATAXIA-TELANGIECTASIA MUTATED) ACTIVATION (opens in a new tab)

  9. Co-producing healthcare guidance for children and young people with ataxia telangiectasia (A-T) including the exploration and design of a home-based complex intervention

    … and young people (CYP) living with ataxia telangiectasia (A-T), a rare and complex neurodegenerative condition. Addressing significant gaps in non-medical care provision, the project was structured into four interrelated work packages (WPs), each contributing original insights and practical …

    plymouth Repository record for Co-producing healthcare guidance for children and young people with ataxia telangiectasia (A-T) including the exploration and design of a home-based complex intervention (opens in a new tab)

  10. Lifestyle and clinical factors related to the deterioration of trunk varicose veins, telangiectasia, chronic venous insufficiency and venous reflux in the general population: Edinburgh Vein Study follow-up

    … when adjusted for gender. The prevalence of telangiectasia was higher in females than in males in both the baseline and follow-up stages of the study (both p<0.01). The rate of deterioration in telangiectasia was 1.6% per annum. The commonest deterioration was from grade I (mild) at baseline …

    edinburgh Repository record for Lifestyle and clinical factors related to the deterioration of trunk varicose veins, telangiectasia, chronic venous insufficiency and venous reflux in the general population: Edinburgh Vein Study follow-up (opens in a new tab)

  11. Identificación macroscópica de patologías hepáticas de mayor prevalencia en bovinos faenados en el matadero PROINCASA, período del 07 de julio del 2010 - enero del 2011

    … afectados con abscesos fue julio con un 5%, telangiectasia en octubre con un 9%, Ictericia en agosto con un 5% y para adherencia fue octubre con un 8%, del total de hígados con diferentes patologías por mes se obtuvo la mayor prevalencia en octubre con un 56% para abscesos, telangiectasia en …

    una Repository record for Identificación macroscópica de patologías hepáticas de mayor prevalencia en bovinos faenados en el matadero PROINCASA, período del 07 de julio del 2010 - enero del 2011 (opens in a new tab)

  12. Double-Strand Break Repair Mechanisms in Human Embryonic Stem Cells

    … kinase (PIKK) family, including Ataxia Telangiectasia Mutated (ATM), Ataxia Telangiectasia Mutated and Rad3-related (ATR) and the DNA dependent protein kinase (DNA-PK). The aim of this study was to define the mechanisms and important proteins involved in repair of human embryonic stem …

    vcu Repository record for Double-Strand Break Repair Mechanisms in Human Embryonic Stem Cells (opens in a new tab)

  13. Diagnostico histopatologico de lesiones hepaticas en bovinos faenados en el rastro municipal de Esteli en el periodo de marzo a agosto de 2008

    … 12% se refiere peri hepatitis, 10% a hepatitis telangiectasia, 5% a hepatitis pigmentación 3.4% hepatitis crónica, hepatitis absceso, peri hepatitis fibrinosa y absceso, y los rangos de 1.7% corresponden a hemorragia, adherencia, telangiectasia absceso, combina 1 y 9, (hepatitis focal crónica, …

    una Repository record for Diagnostico histopatologico de lesiones hepaticas en bovinos faenados en el rastro municipal de Esteli en el periodo de marzo a agosto de 2008 (opens in a new tab)

  14. The role of ATM responding to DNA damage induced by Xrcc1 deficiency during oligodendrocyte genesis

    Ataxia telangiectasia (A-T) is a prime example of hereditary disease about DNA damage signaling and repair protein deficiency. A-T is an autosomal recessive neurodegenerative disorder associated the defective ATM (Ataxia telangiectasia mutated) gene that is estimated to affect 1 in 40,000-300,000 …

    ajou Repository record for The role of ATM responding to DNA damage induced by Xrcc1 deficiency during oligodendrocyte genesis (opens in a new tab)

  15. Tyrosine 370 Phosphorylation of Atm Positively Regulates Dna Damage Response

    <p>Ataxia telangiectasia-mutated (ATM) mediates DNA damage response by controlling irradiation (IR)-induced foci formation, cell cycle checkpoint, and apoptosis. However, how upstream signaling regulates ATM is not completely understood. Here, we show that upon IR stimulation, ATM associates with …

    uthsc Repository record for Tyrosine 370 Phosphorylation of Atm Positively Regulates Dna Damage Response (opens in a new tab)

  16. Distinct DNA Damage Signaling in the Brain Distinguishes ATLD, NBS, and ATR-Seckel Syndrome

    … and the pathway in which it operates. Ataxia-telangiectasia (A-T) is the prototypical disease associated with DNA double strand break (DSB) repair deficiency and is characterized by severe neural pathology. A-T results from homozygous mutations that inactivate the ataxia-telangiectasia mutated …

    tenn-hsc Repository record for Distinct DNA Damage Signaling in the Brain Distinguishes ATLD, NBS, and ATR-Seckel Syndrome (opens in a new tab)

  17. N-linked glycosylation at position ASN98 of the ALK1 receptor protein: relevance for ALK1 function and HHT pathogenesis

    Hereditary Hemorrhagic Telangiectasia (HHT) is an autosomal dominant genetic disorder that results from a mutation of one of two key signaling receptors for the transforming growth factor beta (TGFβ) superfamily: endoglin and activin receptor-like kinase 1 (ALK1). These mutations result in …

    bu Repository record for N-linked glycosylation at position ASN98 of the ALK1 receptor protein: relevance for ALK1 function and HHT pathogenesis (opens in a new tab)

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