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Showing 1 to 13 of 13 for “"optic atrophy"”.
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The Mitochondrial Fusion Protein, Optic Atrophy 1 (OPA1), Alters Cellular Metabolism to Support Cancer Cell Viability
Triple negative breast cancer (TNBC) is widely recognized to be aggressive and difficult to treat due to a lack of targetable hormone receptors. We have become interested in whether TNBC is critically dependent upon alterations in mitochondrial-governed metabolism. To preserve homeostasis, …
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Role and regulation of mitochondrial permeability transition in cell death
… mitochondrial release of the profusion GTPase Optic Atrophy 1 (OPA1), which controls CJ integrity, and cytochrome c leading to apoptosis. Interference RNA knockdown of Bax blocked OPA1 and cytochrome c release after THG treatment, but did not prevent the MPT showing that Bax was essential for …
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ΕΠΙΔΡΑΣΗ ΤΗΣ ΔΙΑΤΟΜΗΣ ΤΟΥ ΟΠΤΙΚΟΥ ΝΕΥΡΟΥ ΕΠΙ ΤΗΣ ΕΝΔΟΦΘΑΛΜΙΟΥ ΠΙΕΣΕΩΣ ΚΑΙ ΔΡΑΣΕΩΣ ΩΣΜΩΤΙΚΩΝ ΚΑΙ ΜΗ ΔΙΟΥΡΗΤΙΚΩΝ ΣΕ ΑΥΤΗΝ
UNILATERAL OPTICAL NERVE TRANSECTION WAS CARRIED OUT RETROBULBARY AND A SHAM PROCEDURE WAS PERFORMED ON THE FELLOW EYE IN RABBITS. BASELINE I.O.P. VALUES, AS WELL AS I.O.P. RESPONSE TO SYSTEMICALLY ADMINISTERED OSMOTIC AGENTS (UREA, MANNITOL AND ASCORBIC ACID), ACETAZOLAMIDE, FUROSEMIDE, NACL O,9% …
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Regulation and roles of nutrient-dependent mitochondrial fusion
… mitochondrial fusion required the regulators, Optic atrophy 1 (Opa1) and Mitofusin 1 (Mfn1).;Metabolomic analysis revealed both QLR-dependent and fusion-dependent changes in levels of metabolites involved in the urea cycle, Krebs cycle and REDOX balance. Findings in this project suggest …
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Evaluation of the visual pathway with ERG, mfERG and mfVEP in inherited eye disorders
… in these patients. Patients with dominant optic atrophy and a known mutation in the OPA-1 gene have a very variable clinical phenotype. MfVEP and ocular blood flow measurements are two new methods for improved identification and characterization of this disorder. A patient with a known …
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Sex Differences in Cancer Cachexia and a Novel Mitochondrial Target for Cancer-Induced Muscle Wasting
… by marked weight loss including skeletal muscle atrophy that affects approximately 80% of cancer patients. Current therapeutic treatments including pharmacological and nutritional intervention are insufficient to prevent or reverse it. Prior studies demonstrated lower muscle mass, impaired muscle …
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Investigating quality of life in inherited optic neuropathies: Evaluating patient experiences and outcome measures
Inherited optic neuropathies (IONs) are a group of rare eye diseases characterised by bilateral and progressive degeneration of the optic nerve due to mitochondrial dysfunction. The two most common IONs encountered in clinical practice are autosomal dominant optic atrophy (DOA) and Leber hereditary …
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Regulation of Mitochondrial Morphology by ERK1/2-Mediated Control of Mfn2 Stability
… 형태는 Mitofusin 1(Mfn1), Mitofusin 2(Mfn2), Optic atrophy protein 1(Opa1), Dynamin related protein 1(Drp1), Fission protein 1(Fis1) 등의 미토콘드리아 형태조절 단백질(mitochondria-shaping protein)이 관여하는 미토콘드리아 융합-분열 기구(mitochondrial fusion-fission machinery)에 의해 정교하게 조절되고 있다. 이러한 형태조절 단백질들이 어떻게 분자 수준에서 조절 되어 …
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A study on the role of oxidative stress and protein kinase signalling in hyperglycaemia induced cardiac remodelling
… of the mitochondrial fusion regulatory protein, optic atrophy-1 (OPA1), with the inhibition of p38MAPK in high glucose attenuating this effect. Conclusion Hyperglycaemia induced pyknotic-like phenomenon, suppressed the proliferation, and reduced mitochondrial fusion protein machinery of …
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Molecular bases, pathogenic mechanisms and possible therapeutic approach in Leber's Hereditary Optic Neuropathy
Leberâ��s hereditary optic neuropathy (LHON) is a mitochondrial disease characterized by a rapid loss of central vision and optic atrophy, due to the selective degeneration of retinal ganglion cells. The age of onset is around 20, and the degenerative process is fast and usually the second eye …
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Mitofusin 2 Regulated Transport of Mitochondria is Necessary for Axonal Integrity
… haplo-insufficiency of opa-1 leads to Dominant Optic Atrophy: DOA) but not degeneration of long peripheral axons, highlighting the potential importance of mitochondrial transport for axon integrity. To further test our hypothesis that mitochondrial transport is critical for the integrity of …
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Mitochondrial dysfunction in hereditary optic neuropathies
MITOCHONDRIAL DYSFUNCTION IN HEREDITARY OPTIC NEUROPATHIES Mitochondrial pathologies are a heterogeneous group of clinical manifestations characterized by oxidative phosphorylation impairment. At the beginning of their recognition mitochondrial pathologies were regarded as rare disorders but indeed …
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Genotype and phenotype in mitochondrial disorders
… most commonly m.3243A>G, Leber Hereditary Optic Neuropathy, and large scale mtDNA rearrangements. The commonest nuclear genetic diagnoses were SPG7, dominant optic atrophy (OPA1) and autosomal recessive POLG. We have linked to Hospital Episode Statistics, death certificates and the cancer …