Sophie Patmore - Mitochondrial Disease - Long Term Ventilation - Tracheostomy
 
LATEST NEWS
01/08/2010 07:37:19
Thank you to 'Stitches of Love and Kindness'
A massive thank you to everyone at 'Stiches of Love and Kindness' who helped to make Sophie's beautiful handmade quilt.
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06/04/2010 19:17:04
Thank you to Jaxson's Blankies for Babes
A special thank you to Jaxson's Blankies for Babes for the special blanket sent to Sophie
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14/12/2009 21:45:16
The Holly Bush launch new website
The Holly Bush country pub based in Frensham launch their own website.
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RECENT BLOGS
01/09/2010 21:37:57
Sophie loves her new train set!
Sophie has lots of fun playing with her new switch adapted Thomas the Tank Engine train set
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16/08/2010 21:51:39
Lots of Water Fun and an Energy Crash
Sophie had a great July with lots of tomboy fun. Unfortunately the end of July and early August bought with it an energy crash.
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MITOCHONDRIAL RSS NEWS FEEDS

MedWorm: Mitochondrial Disease

Maternal obesity and fetal metabolic programming: a fertile epigenetic soil
Acute, Reversible Axonal Energy Failure During Stroke-Like Episodes in MELAS
Genome-wide mapping of nuclear mitochondrial DNA sequences links DNA replication origins to chromosomal double-strand break formation in Schizosaccharomyces pombe [RESEARCH]
High-throughput profiling of amino acids in strains of the Saccharomyces cerevisiae deletion collection [METHOD]
Thyroid hormones, mitochondrial bioenergetics and lipid handling
A Single Mutation in Human Mitochondrial DNA Polymerase Pol {gamma}A Affects Both Polymerization and Proofreading Activities of Only the Holoenzyme [Dna and Chromosomes]
Neurons Efficiently Repair Glutamate-induced Oxidative DNA Damage by a Process Involving CREB-mediated Up-regulation of Apurinic Endonuclease 1 [Molecular Bases Of Disease]
Human Equilibrative Nucleoside Transporter-3 (hENT3) Spectrum Disorder Mutations Impair Nucleoside Transport, Protein Localization, and Stability [Membrane Biology]
Respiration-dependent H2O2 Removal in Brain Mitochondria via the Thioredoxin/Peroxiredoxin System [Molecular Bases Of Disease]
Effects of a novel cystine-based glutathione precursor on oxidative stress in vascular smooth muscle cells
Ca2+ overload and mitochondrial permeability transition pore activation in living {delta}-sarcoglycan-deficient cardiomyocytes
[Zaburzenia genetycznych struktur mitochondrialnych i procesów energetycznych oraz ich rola w etiologii choroby niedokrwiennej serca.]
BAS/BSCR12 Proteomic analysis of smooth muscle cells derived from carotid plaque shows differences between symptomatic and asymptomatic plaques
BAS/BSCR45 The mitochondrial permeability transition pore as a target for cardioprotection in ventricular cardiomyocytes harvested from patients with obstructive hypertrophic cardiomyopathy
FC3 Changes in nuclear and mitochondrial DNA damage in primary vascular smooth muscle derived cells and tissue
Successful left hemihepatectomy and perioperative management of a patient with biliary cystadenocarcinoma, complicated with MELAS syndrome: Report of a case
Mitochondrial abnormalities in the putamen in Parkinson’s disease dyskinesia
Common Genetic Variants of the Mitochondrial Trafficking System and Mitochondrial Uncoupling Proteins Affect the Development of Two Slowly Developing Demyelinating Disorders, Leukoaraiosis and Multiple Sclerosis.
Insulin resistance syndrome blunts the mitochondrial anabolic response following resistance exercise
Low doses of 3-nitropropionic acid in vivo induce damage in mouse skeletal muscle
The burden of inherited leukodystrophies in children
The role of mitochondria in pulmonary vascular remodeling
Choroid plexus failure in the Kearns-Sayre syndrome
Caloric restriction and heart function: is there a sensible link?
Metals, oxidative stress and neurodegenerative disorders.
S-nitroso-N-acetylcysteine ameliorates ischemia-reperfusion injury in the steatotic liver
Nicotinamide improves motor deficits and upregulates PGC-1alpha and BDNF gene expression in a mouse model of Huntington's disease.
Corrigendum to 'Prevalence and natural history of heart disease in adults with primary mitochondrial respiratory chain disease' [Eur J Heart Fail 2010;12:114-121]
Frataxin and Mitochondrial FeS Cluster Biogenesis [Cell Biology]
Mitochondrial biogenesis in the metabolic syndrome and cardiovascular disease
Crystal structure of the α6β6 holoenzyme of propionyl-coenzyme A carboxylase
DNA methylation status of nuclear-encoded mitochondrial genes underlies the tissue-dependent mitochondrial functions
BAX/BCL-XL gene expression ratio inversely correlates with disease progression in chronic myeloid leukemia.
MCM3AP, a novel HBV integration site in hepatocellular carcinoma and its implication in hepatocarcinogenesis
Mitochondria and aging in the vascular system
Mitochondrial disease: recognising more than just the tip of the iceberg.
Role of (−)-epigallocatechin-3-gallate in cell viability, lipogenesis, and retinol-binding protein 4 expression in adipocytes
Mitochondrial Complex II Prevents Hypoxic but Not Calcium- and Proapoptotic Bcl-2 Protein-induced Mitochondrial Membrane Potential Loss [Metabolism]
Regulation of Prostate Cancer Cell Survival by Protein Kinase C{epsilon} Involves Bad Phosphorylation and Modulation of the TNF{alpha}/JNK Pathway [Molecular Bases Of Disease]
Wallerian-like axonal degeneration in the optic nerve after perikaryal excitotoxic insult: an ultrastructural study
Tumor necrosis factor inhibitors as novel therapeutic tools for vascular remodeling diseases.
AMPK-associated signaling to bridge the gap between fuel metabolism and hepatocyte viability.
Cancer type-specific modulation of mitochondrial haplogroups in breast, colorectal and thyroid cancer
Common Inherited Variation in Mitochondrial Genes Is Not Enriched for Associations with Type 2 Diabetes or Related Glycemic Traits
Transmission of Mitochondrial DNA Diseases and Ways to Prevent Them
Mitochondrial base excision repair in mouse synaptosomes during normal aging and in a model of Alzheimer's disease.
Oxidative Stress and Aminopeptidases in Parkinson's Disease Patients with and without Treatment.
DNA Damage Links Mitochondrial Dysfunction to Atherosclerosis and the Metabolic Syndrome.
Mitochondria as integrators of signal transduction and energy production in cardiac physiology and disease
Effects of mitochondrial dysfunction on the immunological properties of microglia
Changes in mitochondrial dynamics during amyloid beta-induced PC12 cell apoptosis.
Licofelone attenuates MPTP-induced neuronal toxicity: behavioral, biochemical and cellular evidence
Clinical Manifestations in Children With Mitochondrial Diseases
Impairment of ATPase, adenine nucleotide translocator, and adenylate kinase causes mitochondrial energy deficit in human skin fibroblasts with chromosome 21 trisomy
Autonomic Symptoms in Carriers of the m.3243A>G Mitochondrial DNA Mutation [Original Contribution]
Mitochondrial DNA transmission, replication and inheritance: a journey from the gamete through the embryo and into offspring and embryonic stem cells
Characterization of neurotoxic effects of NMDA and the novel neuroprotection by phytopolyphenols in mice.
Who wants to live for ever?
Med Sci Monit 2010; 16(9):CR427-432 "The sequence polymorphism of MnSOD gene in subjects with respiratory insufficiency in COPD"
Salvianolic Acid B Protects SH-SY5Y Neuroblastoma Cells from 1-Methyl-4-phenylpyridinium-Induced Apoptosis.
Alpha-synuclein aggregation is involved in the toxicity induced by ferric iron to SK-N-SH neuroblastoma cells
Altered Renal Expression of Angiotensin II Receptors, Renin Receptor, and ACE-2 Precede the Development of Renal Fibrosis in Aging Rats.
Exploring mechanisms of resistance to respiratory inhibitors in field strains of Botrytis cinerea, the causal agent of gray mold.
Monoamine Oxidase Inhibitors as Neuroprotective Agents in Age-Dependent Neurodegenerative Disorders.
Cell Death Targets and Potential Modulators in Alzheimer's Disease.
Genome-wide mapping of nuclear mitochondrial DNA sequences links DNA replication origins to chromosomal double-strand break formation in Schizosaccharomyces pombe [RESEARCH]
Bcl-2 Family Proteins as Therapeutic Targets.
Mitochondria-Targeted Antioxidant Peptides.
Balance is the challenge – The impact of mitochondrial dynamics in Parkinson's disease
Mitochondrial dysfunction in glaucoma and emerging bioenergetic therapies.
Autoantibodies for gastrointestinal organ-specific autoimmune diseases in rheumatoid arthritis patients and their relatives
Preserved motor axonal membrane potential in mitochondrial disease
Cardiolipin Remodeling by ALCAT1 Links Oxidative Stress and Mitochondrial Dysfunction to Obesity.
How neuroglobin protects against Alzheimer's
Clinical application of multiphoton tomography in combination with high-frequency ultrasound for evaluation of skin diseases
Tocotrienols have potent antifibrogenic effects in human intestinal fibroblasts
Sulfur amino acids deficiency caused by grass pea diet plays an important role in the toxicity of L-beta-ODAP by increasing the oxidative stress: studies on a motor neuron cell line.
Scientists discover how neuroglobin protects against Alzheimer's
A polyphenol extract modifies quantity but not quality of liver fatty acid content in high-fat-high-sucrose diet-fed rats: possible implication of the sirtuin pathway.
Transglutaminase 2 inhibits apoptosis induced by calcium-overload through down-regulation of Bax.
174th ENMC International Workshop: Applying pre-implantation genetic diagnosis to mtDNA diseases: Implications of scientific advances 19–21 March 2010, Naarden, The Netherlands
Expression of inflammation-associated genes in circulating leukocytes collected from horses with gastrointestinal tract disease.
Engineered Zinc Finger Proteins for Manipulation of the Human Mitochondrial Genome
Mitochondrial biology in Alzheimer’s disease pathogenesis
Parkinson's disease: mitochondrial molecular pathology, inflammation, statins, and therapeutic neuroprotective nutrition.
PPARγ and chronic kidney disease
Alzheimer's disease: diverse aspects of mitochondrial malfunctioning.
Metabolic manipulators: a well founded strategy to combat mitochondrial dysfunction
Perinatal Tobacco Smoke Exposure Increases Vascular Oxidative Stress and Mitochondrial Damage in Non-Human Primates.
High-throughput profiling of amino acids in strains of the Saccharomyces cerevisiae deletion collection [METHOD]
Effects of the black tea polyphenol theaflavin-2 on apoptotic and inflammatory pathways in vitro and in vivo
ANG II promotes autophagy in podocytes
Autophagy in health and disease. 5. Mitophagy as a way of life
The molecular identity of the mitochondrial Ca2+ sequestration system
Rundataxin, a novel protein with RUN and diacylglycerol binding domains, is mutant in a new recessive ataxia
Global gene expression profiling of somatic motor neuron populations with different vulnerability identify molecules and pathways of degeneration and protection
Dihydrolipoamide dehydrogenase (DLD) deficiency in a Spanish patient with myopathic presentation due to a new mutation in the interface domain
Phylogeography and Population Genetic Structure of the Great Leaf-Nosed Bat (Hipposideros armiger) in China
Alterations in mitochondrial function and spermatozoal motility in goat spermatozoa following incubation with a human lysozyme plasmid.
Protein oxidation in Huntington disease affects energy production and vitamin B6 metabolism.
Morphological and Functional Abnormalities in Mitochondria Associated with Synaptic Degeneration in Prion Disease.
Cytotoxicity of Berberine on human cervical carcinoma HeLa cells through mitochondria, death receptor and MAPK pathways, and in-silico drug-target prediction.
Suppression of oxidative stress and pro-inflammatory mediators by Cymbopogon citratus D. Stapf extract in lipopolysaccharide stimulated murine alveolar macrophages.
Inhibition of transglutaminase 2 mitigates transcriptional dysregulation in models of Huntington disease
Neuroprotective mechanisms of brain-derived neurotrophic factor against 3-nitropropionic acid toxicity: therapeutic implications for Huntington's disease
Physiological functions of mitochondrial fusion
A role for the CISD2 gene in lifespan control and human disease
Manganese superoxide dismutase versus p53: the mitochondrial center
Animal and human studies with the mitochondria-targeted antioxidant MitoQ
Metabolic analysis of 13C-labeled pyruvate for noninvasive assessment of mitochondrial function
Mitochondrial respiratory dysfunction-elicited oxidative stress and posttranslational protein modification in mitochondrial diseases
Potential therapeutic applications of tetrahydrobiopterin: from inherited hyperphenylalaninemia to mitochondrial diseases
The spectrum of autoantibodies in IPEX syndrome is broad and includes anti-mitochondrial autoantibodies.
Ability to delay neuropathological events associated with astrocytic MAO-B increase in a Parkinsonian mouse model: implications for early intervention on disease progression.
Mitochondrial function is altered in articular chondrocytes of an endemic osteoarthritis, Kashin–Beck disease
Human Immunodeficiency Virus Treatment–Induced Adipose Tissue Pathology and Lipoatrophy: Prevalence and Metabolic Consequences
Protection of dopaminergic cells from MPP(+)-Mediated toxicity by histone deacetylase inhibition.
Teaching NeuroImages: Diffuse posterior leukoencephalopathy in MELAS without stroke-like episodes
A hereditary moyamoya syndrome with multisystemic manifestations
Initial Experiences with Proton MR Spectroscopy in Treatment Monitoring of Mitochondrial Encephalopathy.
[Review] A neurological perspective on mitochondrial disease
Protein aggregation in Parkinson's disease.
Biophysical investigation on left ventricular myocytes in rats with experimentally induced diabetes.
Late-onset Leber hereditary optic neuropathy mimicking Susac’s syndrome
Neuronal localization of the mitochondrial protein NIPSNAP1 in rat nervous system
Alveolar Epithelial Cell Injury Due to Zinc Oxide Nanoparticle Exposure.
Toxicological and Pathophysiological Roles of Reactive Oxygen and Nitrogen Species.
The protein NOD2: an innate receptor more complex than previously assumed.
Postconditioning and protection from reperfusion injury: where do we stand? * Position Paper from the Working Group of Cellular Biology of the Heart of the European Society of Cardiology
Wnt signaling regulates mitochondrial physiology and insulin sensitivity [Research Papers]
Insulin signaling meets mitochondria in metabolism.
Dynamic simulation of cardiolipin remodeling: greasing the wheels for an interpretative approach to lipidomics [Research Articles]
Maternal Obesity and Fetal Metabolic Programming: A Fertile Epigenetic Soil.
Dimebon Inhibits Calcium-Induced Swelling of Rat Brain Mitochondria But Does Not Alter Calcium Retention or Cytochrome C Release
Mitophagy in yeast: actors and physiological roles
Mitochondrial Respiratory Chain Dysfunction in Muscle From Patients With Amyotrophic Lateral Sclerosis [Original Contribution]
High fat diet-induced liver steatosis promotes an increase in liver mitochondrial biogenesis in response to hypoxia.
Optical imaging of mitochondrial function uncovers actively propagating waves of mitochondrial membrane potential collapse across intact heart
Novel protein being tested as potential target in Alzheimer's treatment
Role of elevated pressure in TRAIL-induced apoptosis in human lung carcinoma cells
Emerging roles of SUMO modification in Arthritis.
Estrogen-related Receptor {gamma} Is a Key Regulator of Muscle Mitochondrial Activity and Oxidative Capacity [Gene Regulation]
Mixed Lineage Kinase-3 Stabilizes and Functionally Cooperates with TRIBBLES-3 to Compromise Mitochondrial Integrity in Cytokine-induced Death of Pancreatic Beta Cells [Molecular Bases Of Disease]
Protective Effects of Chunghyuldan against ROS-mediated Neuronal Cell Death in Models of Parkinson's Disease
Functional splicing assay supporting that c.70 + 5G > A mutation in the MPV17 gene is disease causing
Mitochondrial Cardiomyopathy Presenting as Hypertrophic Cardiomyopathy With Advanced Chronic Kidney Disease
Bilberry extract protect restraint stress-induced liver damage through attenuating mitochondrial dysfunction.
High-throughput profiling of amino acids in strains of the Saccharomyces cerevisiae deletion collection [METHODS AND RESOURCES]
Melatonin induces pro-apoptotic signaling pathway in human pancreatic carcinoma cells (PANC-1)

Recent Mitochondria News

Energy for Life walkathon in St. Chalres to help find cure - Chicago Daily Herald
'The Sound Of A Snail': A Patient's Greatest Comfort - NPR
Diagram of a mitochondrion, like those used in the mitochondria biofuel cell - Gizmag
Early human 'squatters' drove cave bears to extinction - BBC News
Soon, recharge laptops and cell phones with soda pop - Sify
Soon, recharge your laptops with soda pop - Hindustan Times
Biofuel cells and automatic laces - Inquirer (blog)
Living Batteries Will Perform Better After A Coke And Snickers Bar - Gizmodo Australia
EPA to Start Reducing Mercury Emissions - Huffington Post (blog)

Recent Mitochondria Publications compiled by Ion Channel Media Group

V Le Verche et al. Is Amyotrophic Lateral Sclerosis a Mitochondrial Channelopathy? Neuron
C Bogner et al. Apoptosis: embedded in membranes. Curr Opin Cell Biol
PH Wanrooij et al. G-quadruplex structures in RNA stimulate mitochondrial transcription termination and primer formation. Proc Natl Acad Sci U S A
B Lemos et al. Epigenetic effects of polymorphic Y chromosomes modulate chromatin components, immune response, and sexual conflict. Proc Natl Acad Sci U S A
LN Ojemyr et al. Functional interactions between membrane-bound transporters and membranes. Proc Natl Acad Sci U S A
R Acin-Perez et al. Regulation of intermediary metabolism by the PKC{delta} signalosome in mitochondria. FASEB J
RJ Aitken et al. Apoptosis and DNA damage in human spermatozoa. Asian J Androl
AM Hancock et al. Population Genetic Analysis of the Uncoupling Proteins Supports a Role for UCP3 in Human Cold Resistance. Mol Biol Evol
S Geisler et al. The PINK1/Parkin-mediated mitophagy is compromised by PD-associated mutations. Autophagy

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