| Activation of RXRalpha exerts cardioprotection through transcriptional upregulation of Ndufs4 in heart failure. | Activation of RXRα exerts cardioprotection through transcriptional upregulation of Ndufs4 in heart failure.
Shao M, Li L, Ma L, Song C, Li W, Zhang Y, Cheng W, Chen Y, Yang Y, Wang Q, Li C, Wang Q, Wang W, Wang Y. | 05/9/2024 |
| NDUFS4 regulates cristae remodeling in diabetic kidney disease. | NDUFS4 regulates cristae remodeling in diabetic kidney disease.
Mise K, Long J, Galvan DL, Ye Z, Fan G, Sharma R, Serysheva II, Moore TI, Jeter CR, Anna Zal M, Araki M, Wada J, Schumacker PT, Chang BH, Danesh FR., Free PMC Article | 03/6/2024 |
| Structural insights into respiratory complex I deficiency and assembly from the mitochondrial disease-related ndufs4[-/-] mouse. | Structural insights into respiratory complex I deficiency and assembly from the mitochondrial disease-related ndufs4(-/-) mouse.
Yin Z, Agip AA, Bridges HR, Hirst J., Free PMC Article | 01/29/2024 |
| Ndufs4 KO mice: A model to study comorbid mood disorders associated with mitochondrial dysfunction. | Ndufs4 KO mice: A model to study comorbid mood disorders associated with mitochondrial dysfunction.
van Rensburg DJ, Lindeque Z, Harvey BH, Steyn SF. | 01/5/2024 |
| NDUFS4 promotes tumor progression and predicts prognosis in gastric cancer. | NDUFS4 promotes tumor progression and predicts prognosis in gastric cancer.
Cheng T, Jiang B, Xu M, Yuan C, Tai M, Wu H, Lu B, Sun P, Jiang X, Zhang X. | 12/3/2022 |
| Microglial response promotes neurodegeneration in the Ndufs4 KO mouse model of Leigh syndrome. | Microglial response promotes neurodegeneration in the Ndufs4 KO mouse model of Leigh syndrome.
Aguilar K, Comes G, Canal C, Quintana A, Sanz E, Hidalgo J., Free PMC Article | 09/24/2022 |
| Noninvasive Ophthalmic Imaging Measures Retinal Degeneration and Vision Deficits in Ndufs4-/- Mouse Model of Mitochondrial Complex I Deficiency. | Noninvasive Ophthalmic Imaging Measures Retinal Degeneration and Vision Deficits in Ndufs4-/- Mouse Model of Mitochondrial Complex I Deficiency.
Avrutsky MI, Lawson JM, Smart JE, Chen CW, Troy CM., Free PMC Article | 08/13/2022 |
| Ndufs4 knockout mouse models of Leigh syndrome: pathophysiology and intervention. | Ndufs4 knockout mouse models of Leigh syndrome: pathophysiology and intervention.
van de Wal MAE, Adjobo-Hermans MJW, Keijer J, Schirris TJJ, Homberg JR, Wieckowski MR, Grefte S, van Schothorst EM, van Karnebeek C, Quintana A, Koopman WJH., Free PMC Article | 04/9/2022 |
| MicroRNA-128 inhibits mitochondrial biogenesis and function via targeting PGC1alpha and NDUFS4. | MicroRNA-128 inhibits mitochondrial biogenesis and function via targeting PGC1α and NDUFS4.
Sharma K, Chandra A, Hasija Y, Saini N. | 03/5/2022 |
| Ndufs4 ablation decreases synaptophysin expression in hippocampus. | Ndufs4 ablation decreases synaptophysin expression in hippocampus.
Shil SK, Kagawa Y, Umaru BA, Nanto-Hara F, Miyazaki H, Yamamoto Y, Kobayashi S, Suzuki C, Abe T, Owada Y., Free PMC Article | 11/13/2021 |
| Hypoxia ameliorates brain hyperoxia and NAD(+) deficiency in a murine model of Leigh syndrome. | Hypoxia ameliorates brain hyperoxia and NAD(+) deficiency in a murine model of Leigh syndrome.
Grange RMH, Sharma R, Shah H, Reinstadler B, Goldberger O, Cooper MK, Nakagawa A, Miyazaki Y, Hindle AG, Batten AJ, Wojtkiewicz GR, Schleifer G, Bagchi A, Marutani E, Malhotra R, Bloch DB, Ichinose F, Mootha VK, Zapol WM., Free PMC Article | 08/21/2021 |
| Metallothionein 1 Overexpression Does Not Protect Against Mitochondrial Disease Pathology in Ndufs4 Knockout Mice. | Metallothionein 1 Overexpression Does Not Protect Against Mitochondrial Disease Pathology in Ndufs4 Knockout Mice.
Miller HC, Louw R, Mereis M, Venter G, Boshoff JD, Mienie L, van Reenen M, Venter M, Lindeque JZ, Domínguez-Martínez A, Quintana A, van der Westhuizen FH. | 08/7/2021 |
| Regional metabolic signatures in the Ndufs4(KO) mouse brain implicate defective glutamate/alpha-ketoglutarate metabolism in mitochondrial disease. | Regional metabolic signatures in the Ndufs4(KO) mouse brain implicate defective glutamate/α-ketoglutarate metabolism in mitochondrial disease.
Johnson SC, Kayser EB, Bornstein R, Stokes J, Bitto A, Park KY, Pan A, Sun G, Raftery D, Kaeberlein M, Sedensky MM, Morgan PG., Free PMC Article | 04/13/2021 |
| NDUFS4 deletion triggers loss of NDUFA12 in Ndufs4(-/-) mice and Leigh syndrome patients: A stabilizing role for NDUFAF2. | NDUFS4 deletion triggers loss of NDUFA12 in Ndufs4(-/-) mice and Leigh syndrome patients: A stabilizing role for NDUFAF2.
Adjobo-Hermans MJW, de Haas R, Willems PHGM, Wojtala A, van Emst-de Vries SE, Wagenaars JA, van den Brand M, Rodenburg RJ, Smeitink JAM, Nijtmans LG, Sazanov LA, Wieckowski MR, Koopman WJH. | 10/31/2020 |
| Effects of clofibrate and KH176 on life span and motor function in mitochondrial complex I-deficient mice. | Effects of clofibrate and KH176 on life span and motor function in mitochondrial complex I-deficient mice.
Frambach SJCM, van de Wal MAE, van den Broek PHH, Smeitink JAM, Russel FGM, de Haas R, Schirris TJJ. | 10/24/2020 |
| mice with a congenital Ndufs4 deficiency or chemically inhibited mitochondrial complex I function have impaired HPV | Impaired hypoxic pulmonary vasoconstriction in a mouse model of Leigh syndrome.
Schleifer G, Marutani E, Ferrari M, Sharma R, Skinner O, Goldberger O, Grange RMH, Peneyra K, Malhotra R, Wepler M, Ichinose F, Bloch DB, Mootha VK, Zapol WM., Free PMC Article | 12/28/2019 |
| hematopoietic AIF-deficient or NDUFS4-deficient mice highlighted the fine-tuning of mitochondrial oxidative phosphorylation in immune cells. | Mitochondrial OXPHOS influences immune cell fate: lessons from hematopoietic AIF-deficient and NDUFS4-deficient mouse models.
Bertaux A, Cabon L, Brunelle-Navas MN, Bouchet S, Nemazanyy I, Susin SA., Free PMC Article | 11/9/2019 |
| the partial absence of complex I sensitizes the myocardium towards ischemia reperfusion injury and that the main source of reactive oxygen species following reperfusion is complex III. | Partial loss of complex I due to NDUFS4 deficiency augments myocardial reperfusion damage by increasing mitochondrial superoxide/hydrogen peroxide production.
Kuksal N, Gardiner D, Qi D, Mailloux RJ. | 04/7/2018 |
| Excitatory synaptic transmission in the parietal association cortex in slices from Ndufs4(KO) animals was hypersensitive to isoflurane compared to control slices. We identified a direct neural circuit between the parietal association cortex and the central thalamus, consistent with a model in which isoflurane sensitivity is mediated by a thalamic signal relayed through excitatory synapses to the parietal association cort | Regional knockdown of NDUFS4 implicates a thalamocortical circuit mediating anesthetic sensitivity.
Ramadasan-Nair R, Hui J, Zimin PI, Itsara LS, Morgan PG, Sedensky MM., Free PMC Article | 12/16/2017 |
| Study showed that the earliest cell loss in complex 1-deficient Ndufs4 mice retinas is retinal bipolar cells at p20, followed by Starburst Amacrine Cells at p24, that precede a rise in inflammatory molecules at p30 and retinal ganglion cell death at p42; results could suggest a mechanism in which the death of bipolar and amacrine cells incite an inflammatory wave that ultimately results in retinal ganglion cell loss. | Bipolar cell reduction precedes retinal ganglion neuron loss in a complex 1 knockout mouse model.
Song L, Yu A, Murray K, Cortopassi G. | 08/12/2017 |
| NDUFS4 deletion affected gene expression following neural differentiation and the potential of the cells to generate beating embryoid bodies. | Deletion of the Complex I Subunit NDUFS4 Adversely Modulates Cellular Differentiation.
Johnson J, Lee W, Frazier AE, Vaghjiani V, Laskowski A, Rodriguez AL, Cagnone GL, McKenzie M, White SJ, Nisbet DR, Thorburn DR, St John JC. | 10/22/2016 |
| Using the Ndufs4 knockout (Ndufs4 KO) mouse, a model of Leigh syndrome, we demonstrate for the first time that protein succination is increased in the brainstem (BS), particularly in the vestibular nucleus. | Succination is Increased on Select Proteins in the Brainstem of the NADH dehydrogenase (ubiquinone) Fe-S protein 4 (Ndufs4) Knockout Mouse, a Model of Leigh Syndrome.
Piroli GG, Manuel AM, Clapper AC, Walla MD, Baatz JE, Palmiter RD, Quintana A, Frizzell N., Free PMC Article | 10/22/2016 |
| Locally insufficient respiration capacity of the nerve terminals may drive focal neurodegeneration in the Ndufs4 knockout mouse model of the Leigh syndrome. | Region-Specific Defects of Respiratory Capacities in the Ndufs4(KO) Mouse Brain.
Kayser EB, Sedensky MM, Morgan PG., Free PMC Article | 07/16/2016 |
| This study demonstrated that Genetic reduction of Ndufs4 function does not lead to loss of dopamine neurons in vivo. | Genetic reduction of mitochondrial complex I function does not lead to loss of dopamine neurons in vivo.
Kim HW, Choi WS, Sorscher N, Park HJ, Tronche F, Palmiter RD, Xia Z., Free PMC Article | 04/30/2016 |
| Reduced adolescent-age spatial learning ability is associated with elevated juvenile-age superoxide levels in complex I mouse mutants. | Reduced adolescent-age spatial learning ability associated with elevated juvenile-age superoxide levels in complex I mouse mutants.
Mayer J, Reichart G, Tokay T, Lange F, Baltrusch S, Junghanss C, Wolkenhauer O, Jaster R, Kunz M, Tiedge M, Ibrahim S, Fuellen G, Köhling R., Free PMC Article | 03/26/2016 |