Disease: Dimauro disease
- A Brief History of Mitochondrial Pathologies
- A myopathy with unusual features caused by PNPLA2 gene mutations
- A new muscle glycogen storage disease associated with glycogenin-1 deficiency
- A novel mouse model that recapitulates adult-onset glycogenosis type 4
- A novel mutation in PNPLA2 leading to neutral lipid storage disease with myopathy
- Administration of a Multi-Strain Probiotic Product to Women in the Perinatal Period Differentially Affects the Breast Milk Cytokine Profile and May Have Beneficial Effects on Neonatal Gastrointestinal Functional Symptoms. A Randomized Clinical Trial
- Adult polyglucosan body disease with GBE1 haploinsufficiency and concomitant frontotemporal lobar degeneration
- Alpha B-Crystallin in Muscle Disease Prevention: The Role of Physical Activity
- Alpha B-crystallin induction in skeletal muscle cells under redox imbalance is mediated by a JNK-dependent regulatory mechanism
- AlphaB-crystallin and breast cancer: role and possible therapeutic strategies
- An intelligent non-invasive system for automated diagnosis of anemia exploiting a novel dataset
- Anti-Oxidant Drugs: Novelties and Clinical Implications in Cerebellar Ataxias
- Association of Prenatal Ultrasonography and Autism Spectrum Disorder
- Attitudes toward prevention of mtDNA-related diseases through oocyte mitochondrial replacement therapy
- Biallelic C1QBP Mutations Cause Severe Neonatal-, Childhood-, or Later-Onset Cardiomyopathy Associated with Combined Respiratory-Chain Deficiencies
- Bilateral Pheochromocytomas in a Patient with Y175C Von Hippel-Lindau Mutation
- Branching enzyme deficiency: expanding the clinical spectrum
- Cerebellar ataxia and severe muscle CoQ10 deficiency in a patient with a novel mutation in ADCK3
- Cerebral metabolic abnormalities in A3243G mitochondrial DNA mutation carriers
- Cisternal cerebrospinal fluid analysis in 24 sheep with chronic coenurosis
- Creation of a S1P Lyase bacterial surrogate for structure-based drug design
- Cytochrome c Oxidase Activity Is a Metabolic Checkpoint that Regulates Cell Fate Decisions During T Cell Activation and Differentiation
- Deep intronic GBE1 mutation in manifesting heterozygous patients with adult polyglucosan body disease
- Defective mitochondrial rRNA methyltransferase MRM2 causes MELAS-like clinical syndrome
- Diagnosis and management of mitochondrial disease: a consensus statement from the Mitochondrial Medicine Society
- Discovery and Optimization of Potent, Selective, and Brain-Penetrant 1-Heteroaryl-1<em>H</em>-Indazole LRRK2 Kinase Inhibitors for the Treatment of Parkinson's Disease
- Dysbiosis and Prematurity: Is There a Role for Probiotics?
- Effectiveness and Safety of a Probiotic-Mixture for the Treatment of Infantile Colic: A Double-Blind, Randomized, Placebo-Controlled Clinical Trial with Fecal Real-Time PCR and NMR-Based Metabolomics Analysis
- ENABLE 2017, the First EUROPEAN PhD and Post-Doc Symposium. Session 4: From Discovery to Cure: The Future of Therapeutics
- Estrogen-Receptor-Positive Breast Cancer in Postmenopausal Women: The Role of Body Composition and Physical Exercise
- Exercise-mediated downregulation of MALAT1 expression and implications in primary and secondary cancer prevention
- FGF21 underlies a hormetic response to metabolic stress in methylmalonic acidemia
- Functional cellular analyses reveal energy metabolism defect and mitochondrial DNA depletion in a case of mitochondrial aconitase deficiency
- Functional tricuspid regurgitation: an underestimated issue
- GBE1-related disorders: Adult polyglucosan body disease and its neuromuscular phenotypes
- Genital allergic contact dermatitis in response to contact to thyme in a sanitary pad
- Genome-wide association analysis identifies a mutation in the thiamine transporter 2 (SLC19A3) gene associated with Alaskan Husky encephalopathy
- Human mitochondrial DNA: roles of inherited and somatic mutations
- Identification of peptides in human Hsp20 and Hsp27 that possess molecular chaperone and anti-apoptotic activities
- Impact commentaries. Subacute necrotising encephalomyelopathy (Leigh's disease; Leigh syndrome)
- Impaired Muscle Mitochondrial Biogenesis and Myogenesis in Spinal Muscular Atrophy
- In vitro effects of Beta-2 agonists on skeletal muscle differentiation, hypertrophy, and atrophy
- Infantile encephaloneuromyopathy and defective mitochondrial translation are due to a homozygous RMND1 mutation
- Integrative network-based approaches identified systems-level molecular signatures associated with gallbladder cancer pathogenesis from gallstone diseases
- Is BRCA mutational status a predictor of platinum-based chemotherapy related hematologic toxicity in high-grade serous ovarian cancer patients?
- Late-onset polyglucosan body myopathy in five patients with a homozygous mutation in GYG1
- Leigh syndrome caused by mitochondrial DNA-maintenance defects revealed by whole exome sequencing
- Level of residual enzyme activity modulates the phenotype in phosphoglycerate kinase deficiency
- Long survival in patients with leigh syndrome and the m.10191T>C mutation in MT-ND3 : a case report and review of the literature
- Longitudinal clinical follow-up of a large family with the R357P Twinkle mutation
- Loss-of-function mutations in MGME1 impair mtDNA replication and cause multisystemic mitochondrial disease
- Macrocytic anemia and mitochondriopathy resulting from a defect in sideroflexin 4
- Macrophage derived TNFα promotes hepatic reprogramming to Warburg-like metabolism
- Management of STEC Gastroenteritis: Is There a Role for Probiotics?
- Metabolic Myoglobinuria
- Metabolic shift underlies recovery in reversible infantile respiratory chain deficiency
- MiR-23-TrxR1 as a novel molecular axis in skeletal muscle differentiation
- Mitochondrial diseases
- Mitochondrial diseases in North America: An analysis of the NAMDC Registry
- Mitochondrial dysfunction as a neurobiological subtype of autism spectrum disorder: evidence from brain imaging
- Mitochondrial encephalomyopathy due to a novel mutation in ACAD9
- MPV17 Mutations Causing Adult-Onset Multisystemic Disorder With Multiple Mitochondrial DNA Deletions
- MR evaluation of encephalic leukoaraiosis in sudden sensorineural hearing loss (SSNHL) patients
- New insights in the field of muscle glycogenoses
- Next-generation sequencing reveals DGUOK mutations in adult patients with mitochondrial DNA multiple deletions
- Onychomadesis secondary to allergic contact dermatitis to tioconazole contained in a nail lacquer: Description of three cases
- Optimization of brain-penetrant picolinamide derived leucine-rich repeat kinase 2 (LRRK2) inhibitors
- Patients' experiences of self-identification, seeking support, and anticipation of potential relapse in multiple sclerosis
- Phosphoglycerate mutase deficiency (glycogen storage disease X) caused by a novel variant in PGAM-M
- Physical activity in the prevention of human diseases: role of epigenetic modifications
- Polyglucosan body myopathy caused by defective ubiquitin ligase RBCK1
- Polyglucosan neurotoxicity caused by glycogen branching enzyme deficiency can be reversed by inhibition of glycogen synthase
- Polyphenolic Fraction from Olive Mill Wastewater: Scale-Up and in Vitro Studies for Ophthalmic Nutraceutical Applications
- Progress in the discovery of small molecule modulators of the Cys-loop superfamily receptors
- Prolactin is Expressed in Uterine Leiomyomas and Promotes Signaling and Fibrosis in Myometrial Cells
- Rationale of Probiotic Supplementation during Pregnancy and Neonatal Period
- Resistance training and redox homeostasis: Correlation with age-associated genomic changes
- Retrospective natural history of thymidine kinase 2 deficiency
- Role of exercise-induced reactive oxygen species in the modulation of heat shock protein response
- Sex Differences in antiaging response to short- and long-term high-intensity interval exercise in rat cardiac muscle: Telomerase activity, total antioxidant/oxidant status
- Sildenafil Counteracts the In Vitro Activation of CXCL-9, CXCL-10 and CXCL-11/CXCR3 Axis Induced by Reactive Oxygen Species in Scleroderma Fibroblasts
- Sildenafil Reduces Expression and Release of IL-6 and IL-8 Induced by Reactive Oxygen Species in Systemic Sclerosis Fibroblasts
- Solid Food Introduction and the Development of Food Allergies
- Steady-state redox status in circulating extracellular vesicles: A proof-of-principle study on the role of fitness level and short-term aerobic training in healthy young males
- Structure-Guided Discovery of Aminoquinazolines as Brain-Penetrant and Selective LRRK2 Inhibitors
- Targeted exome sequencing identified a novel frameshift variant in the PGAM2 gene causing glycogen storage disease type X
- Targeting proximal tubule mitochondrial dysfunction attenuates the renal disease of methylmalonic acidemia
- Targeting the Metabolic Enzyme PGAM2 Overcomes Enzalutamide Resistance in Castration-Resistant Prostate Cancer by Inhibiting BCL2 Signaling
- Telomere length is independently associated with age, oxidative biomarkers, and sport training in skeletal muscle of healthy adult males
- The Beneficial Role of Physical Exercise on Anthracyclines Induced Cardiotoxicity in Breast Cancer Patients
- The clinical maze of mitochondrial neurology
- The North American mitochondrial disease registry
- The Phosphodiesterase Type 5 Inhibitor Sildenafil Improves DNA Stability and Redox Homeostasis in Systemic Sclerosis Fibroblasts Exposed to Reactive Oxygen Species
- The Preventive Role of Physical Activity in Systemic Sclerosis: A Cross-Sectional Study on the Correlation with Clinical Parameters and Disease Progression
- The role of αB-crystallin in skeletal and cardiac muscle tissues
- Time to harmonize mitochondrial syndrome nomenclature and classification: A consensus from the North American Mitochondrial Disease Consortium (NAMDC)
- TK2 mutation presenting as indolent myopathy
- What is influencing the phenotype of the common homozygous polymerase-γ mutation p.Ala467Thr?
- Whole exome sequencing identifies a homozygous POLG2 missense variant in an infant with fulminant hepatic failure and mitochondrial DNA depletion
- Whole-exome sequencing detects <em>PYGM</em> variants in two adults with McArdle disease