mitochondrial disease model mouse
Direct regulation of complex I by mitochondrial MEF2D is disrupted in a mouse model of Parkinson disease and in human patients
... 2). Mitochondrial dysfunction and the ensuing oxidative stress cause damages to key cellular macromolecules, including DNA, which affects basic biological processes ranging from bioenergetics to gene transcrip- ...
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[18F]-BMS-747158-02PET imaging for evaluating hepatic mitochondrial complex 1dysfunction in a mouse model of non-alcoholic fatty liver disease
... detect mitochondrial dysfunction in mice fed a methionine- and choline-deficient (MCD) diet, a commonly used NAFLD model ...on mitochondrial complex-1 (MC-1) activity, which is the first component of ...
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Mitochondrial content is preserved throughout disease progression in the mdx mouse model of Duchenne muscular dystrophy, regardless of taurine supplementation
... taurine synthesis improves skeletal muscle function in the mdx mouse model for Duchenne muscular 587. dystrophy[r] ...
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Early Mechanisms of Retinal Degeneration in the harlequin Mouse
... early disease mechanisms leading to their onset and progression remain poorly ...harlequin mouse is a model of human mitochondrial dysfunction and parainflammation leading to subsequent ...
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Blocking the mitochondrial apoptotic pathway preserves motor neuron viability and function in a mouse model of amyotrophic lateral sclerosis
... the mouse CNS of BCL2-associated X protein (BAX) and BCL2-homologous antagonist/killer (BAK), 2 proapoptotic BCL-2 family proteins that together represent an essential gateway to the mitochondrial apoptotic ...
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A mitochondrial bioenergetic etiology of disease
... the mouse of an ND6 G13997A (P25L) mtDNA mutation, which is functionally equivalent to the human ND6 G14600A (P25L) mutation reported to cause optic atrophy when heteroplasmic and Leigh syndrome when homoplasmic ...
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IRS2 increases mitochondrial dysfunction and oxidative stress in a mouse model of Huntington disease
... Immunoprecipitation and Western blot. Immunoprecipitation and immu- noblotting were performed as described previously (40). Antibodies were as follows: rabbit anti-FoxO1, pThr24-FoxO1, Akt, IR, and tubulin (Cell Sig- ...
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MH84 improves mitochondrial dysfunction in a mouse model of early Alzheimer’s disease
... Alzheimer’s disease (AD) is an age-related neurodegen- erative disease and the primary cause for dementia in the elderly which represents a growing public health issue ...proven disease-modifying ...
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Rapamycin enhances survival in a Drosophila model of mitochondrial disease
... Pediatric mitochondrial disorders are a devastating category of diseases caused by deficiencies in mitochondrial ...defective mitochondrial function, and that pharmacological inhibition of TOR by ...
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Ladwig, Anne (2017): Morphologische Analyse der distalen Auswirkungen des axonalen Transportdefizits in einem Tiermodell der Multiplen Sklerose. Dissertation, LMU München: Medizinische Fakultät
... in mitochondrial structure (Baloh, Schmidt, Pestronk, & Milbrandt, 2007; ...cause mitochondrial fragmentation (Fransson, Ruusala, & Aspenström, 2006; ...and mitochondrial aggregates in ...
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Mitochondrial Deficiencies and Oxidative Stress in Parkinson's Disease: A Slippery Slope to Cell Death
... leaky mitochondrial membrane generates increased free ...including mitochondrial respiratory failures must be engendered to understand how radicals are produced and ...
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Mitochondrial neurogastrointestinal encephalopathy: a clinicopathological mimic of Crohn’s disease
... The family described are notable for three reasons. Firstly, both sisters initially had a working diagnosis of Crohn’s disease. Secondly, both sisters remain ambulant and in employment in their late 20s and 30s, ...
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Mitochondrial CaMKII inhibition in airway epithelium protects against allergic asthma
... earlier model of extramitochondrial CaMKII inhibition in airway epithelium (8), our data suggest subcellular specificity to CaMKII signaling in allergic asthma ...our model strengthens our conclusion that ...
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Syntaphilin controls a mitochondrial rheostat for proliferation motility decisions in cancer
... Cell culture. Human glioblastoma (LN229, U251, and U87), prostate adenocarcinoma (LNCaP, C4-2B, DU145, and PC3), breast epithelial (MCF10A), breast adenocarcinoma (MCF7, Hs578T, and MDA-MB-231), lung adenocarcinoma (A549 ...
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Inhibiting mitochondrial respiration prevents cancer in a mouse model of Li Fraumeni syndrome
... of mitochondrial respiration in a mouse model of LFS can markedly improve cancer-free survival in association with inhibition of the aberrant proliferation signaling caused by p53 mutation (Figure ...
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Dantrolene is neuroprotective in Huntington's disease transgenic mouse model
... in mouse models of spinocer- ebellar ataxia type 2 (SCA2) and type 3 (SCA3) [18,19], attenuating glutamate-induced apoptosis of cultured SCA2-58Q Purkinje ...
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Original Article Curcumin improves learning and memory ability via inhibiting activated microglia-mediated inflammation in mouse models of Alzheimer’s disease
... on mouse learning and memory abilities in the model of Alzheimer’s disease (AD), AD mouse models were established by intracranial injection of AlCl3 and 80 male mice were randomly divided into ...
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Eosinophil activation in a mouse model of allergic airways disease
... XII ABBREVIATIONS AHR airways hyperreactivity APC antigen presenting cell BALF bronchoalveolar lavage fluid BSA bovine serum albumin CCR C-C chemokine receptor cDNA complementary DNA Cfe[r] ...
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The effects of chronic simvastatin treatment on the expression of behavioral symptoms in a transgenic mouse model of Huntington’s disease
... Huntington’s disease (HD) is a heritable, neurodegenerative disorder characterized by motor, cognitive, and psychiatric disturbances. An unstable CAG expansion within the gene normally encoding for the Huntingtin ...
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Effects of Rhes Prenylation on Mouse Cognition in a 3-Nitropropionic Acid Animal Model of Huntington's Disease
... movements of the voluntary muscles of the face and extremities. This often leads to displays of unusual facial grimaces, chorea, dystonia, unsteady gait, lack of coordination, and difficulty with speech and swallowing. ...
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