Disease: Mitochondrial complex I deficiency
- 5-Aminolevulinic acid bypasses mitochondrial complex I deficiency and corrects physiological dysfunctions in Drosophila
- A cryptic pathogenic NDUFV1 variant identified by RNA-seq in a patient with normal complex I activity in muscle and transient magnetic resonance imaging changes
- A Drosophila model of mitochondrial disease phenotypic heterogeneity
- A Leigh syndrome caused by compound heterozygous mutations on NDUFAF5 induce early infant death: A case report
- A Novel Mutation Associated with Neonatal Lethal Cardiomyopathy Leads to an Alternative Transcript Expression in the X-Linked Complex I <em>NDUFB11</em> Gene
- A Novel, NADH-Dependent Acrylate Reductase in Vibrio harveyi
- Abnormal morphology and function in retinal ganglion cells derived from patients-specific iPSCs generated from individuals with Leber's hereditary optic neuropathy
- Acute myocardial infarction in a patient with MELAS syndrome: a possible link?
- Adenosine Awakens Metabolism to Enhance Growth-Independent Killing of Tolerant and Persister Bacteria across Multiple Classes of Antibiotics
- Adipose Mitochondrial Complex I Deficiency Modulates Inflammation and Glucose Homeostasis in a Sex-Dependent Manner
- AGK regulates the progression to NASH by affecting mitochondria complex I function
- Biallelic Loss-of-Function <em>NDUFA12</em> Variants Cause a Wide Phenotypic Spectrum from Leigh/Leigh-Like Syndrome to Isolated Optic Atrophy
- Case report of atypical Leigh syndrome in an adolescent male with novel biallelic variants in NDUFAF5 and review of the natural history of NDUFAF5-related disorders
- Case report: malignant hypertension associated with catecholamine excess in a patient with Leigh syndrome
- Case report: Novel <em>SCN4A</em> variant associated with a severe congenital myasthenic syndrome/myopathy phenotype
- Case report: novel mutations of NDUFS6 and NHLRC2 genes potentially cause the quick postnatal death of a Chinese Hani minority neonate with mitochondrial complex I deficiency and FINCA syndrome
- Combined Transcriptome and Metabolome Analysis Reveals That the Potent Antifungal Pyrylium Salt Inhibits Mitochondrial Complex I in Candida albicans
- Compound heterozygous mutations of NDUFV1 identified in a child with mitochondrial complex I deficiency
- Continuous Hypoxia Reduces Retinal Ganglion Cell Degeneration in a Mouse Model of Mitochondrial Optic Neuropathy
- Corrigendum: A Novel Variation in the Mitochondrial Complex I Assembly Factor NDUFAF5 Causes Isolated Bilateral Striatal Necrosis in Childhood
- CPEB1, a novel risk gene in recent-onset schizophrenia, contributes to mitochondrial complex I defect caused by a defective provirus ERVWE1
- Cross-comparison of systemic and tissue-specific metabolomes in a mouse model of Leigh syndrome
- De novo frameshift variant in MT-ND1 causes a mitochondrial complex I deficiency associated with MELAS syndrome
- Deficiency in coatomer complex I causes aberrant activation of STING signalling
- Deficiency of the mitochondrial ribosomal subunit, MRPL50, causes autosomal recessive syndromic premature ovarian insufficiency
- Differential mitochondrial and cellular responses between H vs. J mtDNA haplogroup-containing human RPE transmitochondrial cybrid cells
- Differential requirements for mitochondrial electron transport chain components in the adult murine liver
- DMT1 differentially regulates mitochondrial complex activities to reduce glutathione loss and mitigate ferroptosis
- Dual Mode of Mitochondrial ROS Action during Reprogramming to Pluripotency
- Early embryonic lethality in complex I associated p.L104P Nubpl mutant mice
- Early Forms of <em>α</em>-Synuclein Pathology Are Associated with Neuronal Complex I Deficiency in the Substantia Nigra of Individuals with Parkinson's Disease
- Early loss of endogenous NAD<sup>+</sup> following rotenone treatment leads to mitochondrial dysfunction and Sarm1 induction that is ameliorated by PARP inhibition
- Effects of Ndufs4 Deletion on Hearing after Various Acoustic Exposures
- Enhanced mitochondrial activity reshapes a gut microbiota profile that delays NASH progression
- Expanding the phenotypic and molecular spectrum of NFS1-related disorders that cause functional deficiencies in mitochondrial and cytosolic iron-sulfur cluster containing enzymes
- Exploration of mitochondrial defects in sarcopenic hip fracture patients
- FDX2 and ISCU Gene Variations Lead to Rhabdomyolysis With Distinct Severity and Iron Regulation
- FTSH PROTEASE 3 facilitates Complex I degradation through a direct interaction with the Complex I subunit PSST
- Generation of a human induced pluripotent stem cell line NTUHi004-A from a patient with Leigh syndrome harboring a homozygous missense mutation c.836 T > G (p.Met279Arg) in NDUFAF5 gene
- Genetic BACH1 deficiency alters mitochondrial function and increases NLRP3 inflammasome activation in mouse macrophages
- Genetic Reduction of Mitochondria Complex I Subunits is Protective against Cisplatin-Induced Neurotoxicity in <em>Drosophila</em>
- Huangqin Decoction Exerts Beneficial Effects on Rotenone-Induced Rat Model of Parkinson's Disease by Improving Mitochondrial Dysfunction and Alleviating Metabolic Abnormality of Mitochondria
- Hypoxia Induces Renal Epithelial Injury and Activates Fibrotic Signaling Through Up-Regulation of Arginase-II
- Identification of a novel MT-ND3 variant and restoring mitochondrial function by allotopic expression of MT-ND3 gene
- Impact of a <em>NDUFC2</em> Variant on the Occurrence of Acute Coronary Syndromes
- Impairments in SHMT2 expression or cellular folate availability reduce oxidative phosphorylation and pyruvate kinase activity
- Inhibition of mitochondrial complex I leading to NAD<sup>+</sup>/NADH imbalance in type 2 diabetic patients who developed late stent thrombosis: Evidence from an integrative analysis of platelet bioenergetics and metabolomics
- Involvement of Mitochondria in Parkinson's Disease
- Iron status influences mitochondrial disease progression in Complex I-deficient mice
- Leber's hereditary optic neuropathy-associated ND6 14484T > C mutation caused pleiotropic effects on the complex I, RNA homeostasis, apoptosis and mitophagy
- Lip cyanosis as the first symptom of Leigh syndrome associated with mitochondrial complex I deficiency due to a compound heterozygous NDUFS1 mutation: A case report
- Long-chain fatty acid oxidation and respiratory complex I deficiencies distinguish Barth Syndrome from idiopathic pediatric cardiomyopathy
- Loss of mitochondrial Chchd10 or Chchd2 in zebrafish leads to an ALS-like phenotype and Complex I deficiency independent of the mitochondrial integrated stress response
- Mangiferin, a natural glucoxilxanthone, inhibits mitochondrial dynamin-related protein 1 and relieves aberrant mitophagic proteins in mice model of Parkinson's disease
- Mesenchymal stem cells improve redox homeostasis and mitochondrial respiration in fibroblast cell lines with pathogenic MT-ND3 and MT-ND6 variants
- Metabolic rescue ameliorates mitochondrial encephalo-cardiomyopathy in murine and human iPSC models of Leigh syndrome
- Microglial response promotes neurodegeneration in the Ndufs4 KO mouse model of Leigh syndrome
- MiR-29a inhibits MPP + - Induced cell death and inflammation in Parkinson's disease model in vitro by potential targeting of MAVS
- Mitochondrial complex I deficiency and Parkinson disease
- Mitochondrial Complex I Deficiency Masquerading as Stroke-Like Episode Clinically and as Alexander Disease Radiologically Following Chicken Pox
- Mitochondrial complex I deficiency stratifies idiopathic Parkinson's disease
- Mitochondrial Complex I deficiency: guilty in Parkinson's disease
- Mitochondrial complex I dysfunction alters the balance of soluble and membrane-bound TNF during chronic experimental colitis
- Mitochondrial complex I subunit deficiency promotes pancreatic α-cell proliferation
- Mitochondrial Respiratory Chain Dysfunction-A Hallmark Pathology of Idiopathic Parkinson's Disease?
- Mitochondrial respiratory complex I deficiency inhibits brown adipogenesis by limiting heme regulation of histone demethylation
- NDUFA1 p.Gly32Arg variant in early-onset dementia
- NDUFC2 deficiency exacerbates endothelial mesenchymal transformation during ischemia-reperfusion via NLRP3
- Ndufs4 knockout mouse models of Leigh syndrome: pathophysiology and intervention
- NDUFV1 mutations in complex I deficiency: Case reports and review of symptoms
- Next generation sequencing reveals novel compound heterozygous deletions in NDUFAF2 in a child with mitochondrial complex I deficiency, nuclear type 10
- Noninvasive Ophthalmic Imaging Measures Retinal Degeneration and Vision Deficits in Ndufs4-/- Mouse Model of Mitochondrial Complex I Deficiency
- Novel biallelic mutations in TMEM126B cause splicing defects and lead to Leigh-like syndrome with severe complex I deficiency
- Optimized allotopic expression of mitochondrial ND6 transgene restored complex I and apoptosis deficiencies caused by LHON-linked ND6 14484T > C mutation
- Pathways controlling neurotoxicity and proteostasis in mitochondrial complex I deficiency
- PHB2 ameliorates Doxorubicin-induced cardiomyopathy through interaction with NDUFV2 and restoration of mitochondrial complex I function
- Phenotypic Heterogeneity in Patients with Mutations in the Mitochondrial Complex I Assembly Gene NDUFAF5
- Polymorphic variants at NDUFC2, encoding a mitochondrial complex I subunit, associate with cardiac hypertrophy in human hypertension
- Rapidly Progressive Spastic Paraplegia Due to Hyperhomocysteinemia in Child with <em>MTHFR</em> Gene Mutation and Mitochondrial Complex I Deficiency: A Rare Association
- Rapidly Progressive Spastic Paraplegia Due to Hyperhomocysteinemia in Child with MTHFR Gene Mutation and Mitochondrial Complex I Deficiency: A Rare Association
- Restoring cellular NAD(P)H levels by PPARalpha and LXRalpha stimulation to improve mitochondrial complex I deficiency
- Restoring cellular NAD(P)H levels by PPARα and LXRα stimulation to improve mitochondrial complex I deficiency
- Rotenone-Induced Model of Parkinson's Disease: Beyond Mitochondrial Complex I Inhibition
- Severe Antenatal Hypertrophic Cardiomyopathy Secondary to <em>ACAD9</em>-Related Mitochondrial Complex I Deficiency
- Severe Antenatal Hypertrophic Cardiomyopathy Secondary to ACAD9-Related Mitochondrial Complex I Deficiency
- SHORT-TERM INFLAMMATORY BIOMARKER PROFILES ARE ASSOCIATED WITH DEFICIENT MITOCHONDRIAL BIOENERGETICS IN LYMPHOCYTES OF SEPTIC SHOCK PATIENTS-A PROSPECTIVE COHORT STUDY
- Skeletal muscle mitochondrial dysfunction in contemporary antiretroviral therapy: a single cell analysis
- Succinate prodrugs as treatment for acute metabolic crisis during fluoroacetate intoxication in the rat
- Targeting Mitochondrial Complex I Deficiency in MPP(+)/MPTP-induced Parkinson's Disease Cell Culture and Mouse Models by Transducing Yeast NDI1 Gene
- The clinically relevant triple mutation in the mtND1 gene inactivates Escherichia coli complex I
- The decylTPP mitochondria-targeting moiety lowers electron transport chain supercomplex levels in primary human skin fibroblasts
- The Mysterious Multitude: Structural Perspective on the Accessory Subunits of Respiratory Complex I
- The Orphan Response Regulator Rv3143 Modulates the Activity of the NADH Dehydrogenase Complex (Nuo) in <em>Mycobacterium tuberculosis via</em> Protein-Protein Interactions
- Transgenic NADH dehydrogenase restores oxygen regulation of breathing in mitochondrial complex I-deficient mice
- Tumor growth of neurofibromin-deficient cells is driven by decreased respiration and hampered by NAD<sup>+</sup> and SIRT3
- Ubiquitinated AIF is a major mediator of hypoxia-induced mitochondrial dysfunction and pulmonary artery smooth muscle cell proliferation
- Unique prenatal manifestations of biallelic NDUFAF5 variants: expansion of phenotype
- Unique prenatal manifestations of biallelic NDUFAF5 variants: expansion of the phenotype
- Variable effects of omaveloxolone (RTA408) on primary fibroblasts with mitochondrial defects
- Yeast NDI1 reconfigures neuronal metabolism and prevents the unfolded protein response in mitochondrial complex I deficiency