The Charcot-Marie-Tooth type 2A gene product, Mfn2, up-regulates fuel oxidation through expression of OXPHOS system.

PubWeight™: 2.72‹?› | Rank: Top 1%

🔗 View Article (PMID 15829499)

Published in Hum Mol Genet on April 13, 2005

Authors

Sara Pich1, Daniel Bach, Paz Briones, Marc Liesa, Marta Camps, Xavier Testar, Manuel Palacín, Antonio Zorzano

Author Affiliations

1: Departament de Bioquímica i Biologia Molecular, Facultat de Biologia, Universitat de Barcelona, and IRBB-PCB, Parc Científic de Barcelona, Barcelona, Spain.

Articles citing this

(truncated to the top 100)

AMPK regulates energy expenditure by modulating NAD+ metabolism and SIRT1 activity. Nature (2009) 10.59

Multi-system neurological disease is common in patients with OPA1 mutations. Brain (2010) 5.37

Nitric oxide-induced mitochondrial fission is regulated by dynamin-related GTPases in neurons. EMBO J (2006) 4.43

Mitochondrial optic neuropathies - disease mechanisms and therapeutic strategies. Prog Retin Eye Res (2010) 3.58

Interdependence of AMPK and SIRT1 for metabolic adaptation to fasting and exercise in skeletal muscle. Cell Metab (2010) 3.46

Mitofusin 2 is necessary for transport of axonal mitochondria and interacts with the Miro/Milton complex. J Neurosci (2010) 2.55

Preventing mitochondrial fission impairs mitochondrial function and leads to loss of mitochondrial DNA. PLoS One (2008) 2.47

Mitofusin-2 maintains mitochondrial structure and contributes to stress-induced permeability transition in cardiac myocytes. Mol Cell Biol (2011) 2.23

Mitochondrial dynamics controlled by mitofusins regulate Agrp neuronal activity and diet-induced obesity. Cell (2013) 2.11

New insights into the role of mitochondria in aging: mitochondrial dynamics and more. J Cell Sci (2010) 2.03

Mitofusin 2 (Mfn2) links mitochondrial and endoplasmic reticulum function with insulin signaling and is essential for normal glucose homeostasis. Proc Natl Acad Sci U S A (2012) 2.00

Repeated transient mRNA bursts precede increases in transcriptional and mitochondrial proteins during training in human skeletal muscle. J Physiol (2010) 1.98

Genome-wide analysis of the H3K4 histone demethylase RBP2 reveals a transcriptional program controlling differentiation. Mol Cell (2008) 1.93

Mitochondrial morphology, topology, and membrane interactions in skeletal muscle: a quantitative three-dimensional electron microscopy study. J Appl Physiol (1985) (2012) 1.74

Mfn2 modulates the UPR and mitochondrial function via repression of PERK. EMBO J (2013) 1.65

Mitochondrial turnover and aging of long-lived postmitotic cells: the mitochondrial-lysosomal axis theory of aging. Antioxid Redox Signal (2010) 1.64

Mitochondrial Morphology and Fundamental Parameters of the Mitochondrial Respiratory Chain Are Altered in Caenorhabditis elegans Strains Deficient in Mitochondrial Dynamics and Homeostasis Processes. PLoS One (2015) 1.53

Disturbed mitochondrial dynamics and neurodegenerative disorders. Nat Rev Neurol (2014) 1.52

Mitochondrial morphology and cardiovascular disease. Cardiovasc Res (2010) 1.51

Mitochondrial fusion is increased by the nuclear coactivator PGC-1beta. PLoS One (2008) 1.50

Mitochondrial dynamics in diabetes. Antioxid Redox Signal (2010) 1.46

Stress-induced phosphorylation and proteasomal degradation of mitofusin 2 facilitates mitochondrial fragmentation and apoptosis. Mol Cell (2012) 1.46

Subjects with early-onset type 2 diabetes show defective activation of the skeletal muscle PGC-1{alpha}/Mitofusin-2 regulatory pathway in response to physical activity. Diabetes Care (2009) 1.42

Mitochondrial energy metabolism and redox signaling in brain aging and neurodegeneration. Antioxid Redox Signal (2012) 1.32

Biophysical properties of mitochondrial fusion events in pancreatic beta-cells and cardiac cells unravel potential control mechanisms of its selectivity. Am J Physiol Cell Physiol (2010) 1.27

Muscle oxidative capacity during IL-6-dependent cancer cachexia. Am J Physiol Regul Integr Comp Physiol (2010) 1.19

Mitochondria: isolation, structure and function. J Physiol (2011) 1.14

Novel role of FATP1 in mitochondrial fatty acid oxidation in skeletal muscle cells. J Lipid Res (2009) 1.12

Early exposure to general anesthesia disturbs mitochondrial fission and fusion in the developing rat brain. Anesthesiology (2013) 1.11

Central role of mitofusin 2 in autophagosome-lysosome fusion in cardiomyocytes. J Biol Chem (2012) 1.10

Mitochondrial dynamics in the adult cardiomyocytes: which roles for a highly specialized cell? Front Physiol (2013) 1.08

Mitochondrial fission in endothelial cells after simulated ischemia/reperfusion: role of nitric oxide and reactive oxygen species. Free Radic Biol Med (2011) 1.06

Novel C12orf65 mutations in patients with axonal neuropathy and optic atrophy. J Neurol Neurosurg Psychiatry (2013) 1.06

PGC1α-mediated mitofusin-2 deficiency in female rats and humans with pulmonary arterial hypertension. Am J Respir Crit Care Med (2013) 1.06

Role of mitofusin 2 (Mfn2) in controlling cellular proliferation. FASEB J (2013) 1.03

Mitochondrial dysfunction and insulin resistance: an update. Endocr Connect (2014) 1.02

IL-6 regulation on skeletal muscle mitochondrial remodeling during cancer cachexia in the ApcMin/+ mouse. Skelet Muscle (2012) 1.02

Insulin stimulates mitochondrial fusion and function in cardiomyocytes via the Akt-mTOR-NFκB-Opa-1 signaling pathway. Diabetes (2013) 1.01

Cerebral involvement in axonal Charcot-Marie-Tooth neuropathy caused by mitofusin2 mutations. J Neurol (2008) 1.01

Mitochondrial morphology transitions and functions: implications for retrograde signaling? Am J Physiol Regul Integr Comp Physiol (2013) 1.01

Physiological consequences of complex II inhibition for aging, disease, and the mKATP channel. Biochim Biophys Acta (2013) 0.99

Coenzyme Q protects Caenorhabditis elegans GABA neurons from calcium-dependent degeneration. Proc Natl Acad Sci U S A (2010) 0.99

Mitochondrial dysregulation in the pathogenesis of diabetes: potential for mitochondrial biogenesis-mediated interventions. Exp Diabetes Res (2011) 0.98

Mitochondrial dynamics in heart failure. Congest Heart Fail (2012) 0.98

Impaired complex IV activity in response to loss of LRPPRC function can be compensated by mitochondrial hyperfusion. Proc Natl Acad Sci U S A (2013) 0.96

Mitofusins are required for angiogenic function and modulate different signaling pathways in cultured endothelial cells. J Mol Cell Cardiol (2011) 0.96

Nitrite activates protein kinase A in normoxia to mediate mitochondrial fusion and tolerance to ischaemia/reperfusion. Cardiovasc Res (2013) 0.93

Ophthalmic manifestations of inherited neurodegenerative disorders. Nat Rev Neurol (2014) 0.92

Mitochondria in heart failure: the emerging role of mitochondrial dynamics. Heart Fail Rev (2013) 0.90

Krüppel-like factor 4 interacts with p300 to activate mitofusin 2 gene expression induced by all-trans retinoic acid in VSMCs. Acta Pharmacol Sin (2010) 0.90

Skeletal muscle mitochondria as a target to prevent or treat type 2 diabetes mellitus. Nat Rev Endocrinol (2016) 0.90

Perspectives on: SGP symposium on mitochondrial physiology and medicine: what comes first, misshape or dysfunction? The view from metabolic excess. J Gen Physiol (2012) 0.90

Mitochondrial metabolism and the control of vascular smooth muscle cell proliferation. Front Cell Dev Biol (2014) 0.89

Effects of short-term endurance exercise training on acute doxorubicin-induced FoxO transcription in cardiac and skeletal muscle. J Appl Physiol (1985) (2014) 0.89

The suppression of ghrelin signaling mitigates age‐associated thermogenic impairment. Aging (Albany NY) (2014) 0.89

The complex interplay between mitochondrial dynamics and cardiac metabolism. J Bioenerg Biomembr (2011) 0.89

HIV-1 Vpr triggers mitochondrial destruction by impairing Mfn2-mediated ER-mitochondria interaction. PLoS One (2012) 0.89

Patients with sepsis exhibit increased mitochondrial respiratory capacity in peripheral blood immune cells. Crit Care (2013) 0.89

Mitochondrial dynamics in cancer and neurodegenerative and neuroinflammatory diseases. Int J Cell Biol (2012) 0.88

Increased mitochondrial function downstream from KDM5A histone demethylase rescues differentiation in pRB-deficient cells. Genes Dev (2015) 0.87

Role of mitofusin 2 in the renal stress response. PLoS One (2012) 0.87

Adaptability to hypobaric hypoxia is facilitated through mitochondrial bioenergetics: an in vivo study. Br J Pharmacol (2013) 0.87

Dengue Virus Impairs Mitochondrial Fusion by Cleaving Mitofusins. PLoS Pathog (2015) 0.86

Mitofusin 2-deficiency suppresses cell proliferation through disturbance of autophagy. PLoS One (2015) 0.85

A nonsense mutation in DHTKD1 causes Charcot-Marie-Tooth disease type 2 in a large Chinese pedigree. Am J Hum Genet (2012) 0.85

Adenine nucleotide translocase is involved in a mitochondrial coupling defect in MFN2-related Charcot-Marie-Tooth type 2A disease. Neurogenetics (2009) 0.84

Exercise training decreases activation of the mitochondrial fission protein dynamin-related protein-1 in insulin-resistant human skeletal muscle. J Appl Physiol (1985) (2014) 0.84

From structure to function: mitochondrial morphology, motion and shaping in vascular smooth muscle. J Vasc Res (2013) 0.84

MISC-1/OGC links mitochondrial metabolism, apoptosis and insulin secretion. PLoS One (2011) 0.83

Mfn2 downregulation in excitotoxicity causes mitochondrial dysfunction and delayed neuronal death. EMBO J (2014) 0.83

Over-expressing mitofusin-2 in healthy mature mammalian skeletal muscle does not alter mitochondrial bioenergetics. PLoS One (2013) 0.83

Metabolic dysfunction and altered mitochondrial dynamics in the utrophin-dystrophin deficient mouse model of duchenne muscular dystrophy. PLoS One (2015) 0.82

miR-27 regulates mitochondrial networks by directly targeting the mitochondrial fission factor. Exp Mol Med (2014) 0.82

Mitochondrial fission is required for cardiomyocyte hypertrophy mediated by a Ca2+-calcineurin signaling pathway. J Cell Sci (2014) 0.82

Mitochondrial dynamics, mitophagy and cardiovascular disease. J Physiol (2016) 0.82

Heart failure and mitochondrial dysfunction: the role of mitochondrial fission/fusion abnormalities and new therapeutic strategies. J Cardiovasc Pharmacol (2014) 0.82

Cytoplasmic signaling in the control of mitochondrial uproar? Cell Commun Signal (2008) 0.81

Nitrite augments glucose uptake in adipocytes through the protein kinase A-dependent stimulation of mitochondrial fusion. Free Radic Biol Med (2014) 0.81

In utero exposure to prepregnancy maternal obesity and postweaning high-fat diet impair regulators of mitochondrial dynamics in rat placenta and offspring. Physiol Genomics (2014) 0.81

Glucocorticoid modulation of mitochondrial function in hepatoma cells requires the mitochondrial fission protein Drp1. Antioxid Redox Signal (2012) 0.81

Emerging concepts in the molecular basis of pulmonary arterial hypertension: part I: metabolic plasticity and mitochondrial dynamics in the pulmonary circulation and right ventricle in pulmonary arterial hypertension. Circulation (2015) 0.81

Nutrient availability links mitochondria, apoptosis, and obesity. Aging (Albany NY) (2012) 0.80

Diet impact on mitochondrial bioenergetics and dynamics. Front Physiol (2015) 0.80

Mitofusin 2 protects hepatocyte mitochondrial function from damage induced by GCDCA. PLoS One (2013) 0.79

The mitochondrial fusion-related proteins Mfn2 and OPA1 are transcriptionally induced during differentiation of bone marrow progenitors to immature dendritic cells. Mol Cells (2014) 0.78

Implications of mitochondrial dynamics on neurodegeneration and on hypothalamic dysfunction. Front Aging Neurosci (2015) 0.78

Increased insulin sensitivity and distorted mitochondrial adaptations during muscle unloading. Int J Mol Sci (2012) 0.78

Defective mitochondrial function and motility due to mitofusin 1 overexpression in insulin secreting cells. Korean J Physiol Pharmacol (2012) 0.78

Mitofusin 2 Deficiency Affects Energy Metabolism and Mitochondrial Biogenesis in MEF Cells. PLoS One (2015) 0.77

Deficiencies in mitochondrial dynamics sensitize Caenorhabditis elegans to arsenite and other mitochondrial toxicants by reducing mitochondrial adaptability. Toxicology (2017) 0.77

The NFL-TBS.40-63 anti-glioblastoma peptide disrupts microtubule and mitochondrial networks in the T98G glioma cell line. PLoS One (2014) 0.76

Energy imbalance alters Ca(2+) handling and excitability of POMC neurons. Elife (2017) 0.75

MLN64 induces mitochondrial dysfunction associated with increased mitochondrial cholesterol content. Redox Biol (2017) 0.75

Mitofusion-2-mediated alleviation of insulin resistance in rats through reduction in lipid intermediate accumulation in skeletal muscle. J Biomed Sci (2013) 0.75

AKAP350C targets to mitochondria via a novel amphipathic alpha helical domain. Cell Logist (2014) 0.75

Quercetin Affects Erythropoiesis and Heart Mitochondrial Function in Mice. Oxid Med Cell Longev (2015) 0.75

Role of Mitochondria-Associated Endoplasmic Reticulum Membrane in Inflammation-Mediated Metabolic Diseases. Mediators Inflamm (2016) 0.75

Mechanism of Activation-Induced Downregulation of Mitofusin 2 in Human Peripheral Blood T Cells. J Immunol (2015) 0.75

The abundance of the ARL2 GTPase and its GAP, ELMOD2, at mitochondria are modulated by the fusogenic activity of mitofusins and stressors. PLoS One (2017) 0.75

In vivo imaging reveals mitophagy independence in the maintenance of axonal mitochondria during normal aging. Aging Cell (2017) 0.75

Articles by these authors

Guidelines for the use and interpretation of assays for monitoring autophagy. Autophagy (2012) 20.08

Telomere dysfunction induces metabolic and mitochondrial compromise. Nature (2011) 6.36

Pancreatic cancers require autophagy for tumor growth. Genes Dev (2011) 6.17

Mitochondrial dynamics in mammalian health and disease. Physiol Rev (2009) 3.59

Mitofusin-2 determines mitochondrial network architecture and mitochondrial metabolism. A novel regulatory mechanism altered in obesity. J Biol Chem (2003) 3.45

Mitofusin 2 in POMC neurons connects ER stress with leptin resistance and energy imbalance. Cell (2013) 2.43

Mitofusin 2 (Mfn2) links mitochondrial and endoplasmic reticulum function with insulin signaling and is essential for normal glucose homeostasis. Proc Natl Acad Sci U S A (2012) 2.00

Cerebellar ataxia with coenzyme Q10 deficiency: diagnosis and follow-up after coenzyme Q10 supplementation. J Neurol Sci (2006) 1.95

Mitofusins 1/2 and ERRalpha expression are increased in human skeletal muscle after physical exercise. J Physiol (2005) 1.94

Antitelomerase therapy provokes ALT and mitochondrial adaptive mechanisms in cancer. Cell (2012) 1.91

Evidence for a mitochondrial regulatory pathway defined by peroxisome proliferator-activated receptor-gamma coactivator-1 alpha, estrogen-related receptor-alpha, and mitofusin 2. Diabetes (2006) 1.91

Expression of Mfn2, the Charcot-Marie-Tooth neuropathy type 2A gene, in human skeletal muscle: effects of type 2 diabetes, obesity, weight loss, and the regulatory role of tumor necrosis factor alpha and interleukin-6. Diabetes (2005) 1.77

Comparison between SLC3A1 and SLC7A9 cystinuria patients and carriers: a need for a new classification. J Am Soc Nephrol (2002) 1.73

CXC ligand 5 is an adipose-tissue derived factor that links obesity to insulin resistance. Cell Metab (2009) 1.68

Mfn2 modulates the UPR and mitochondrial function via repression of PERK. EMBO J (2013) 1.65

Coenzyme Q deficiency triggers mitochondria degradation by mitophagy. Autophagy (2009) 1.65

Increased ER-mitochondrial coupling promotes mitochondrial respiration and bioenergetics during early phases of ER stress. J Cell Sci (2011) 1.65

Changes in mitochondrial dynamics during ceramide-induced cardiomyocyte early apoptosis. Cardiovasc Res (2007) 1.63

dDOR is an EcR coactivator that forms a feed-forward loop connecting insulin and ecdysone signaling. Curr Biol (2010) 1.62

Loss of mitochondrial protease OMA1 alters processing of the GTPase OPA1 and causes obesity and defective thermogenesis in mice. EMBO J (2012) 1.58

A fatal mitochondrial disease is associated with defective NFU1 function in the maturation of a subset of mitochondrial Fe-S proteins. Am J Hum Genet (2011) 1.57

X-linked NDUFA1 gene mutations associated with mitochondrial encephalomyopathy. Ann Neurol (2007) 1.53

Mitochondrial fusion is increased by the nuclear coactivator PGC-1beta. PLoS One (2008) 1.50

Mitochondrial fusion proteins: dual regulators of morphology and metabolism. Semin Cell Dev Biol (2010) 1.44

Subjects with early-onset type 2 diabetes show defective activation of the skeletal muscle PGC-1{alpha}/Mitofusin-2 regulatory pathway in response to physical activity. Diabetes Care (2009) 1.42

A new inborn error of glycosylation due to a Cog8 deficiency reveals a critical role for the Cog1-Cog8 interaction in COG complex formation. Hum Mol Genet (2007) 1.40

Pathophysiology and treatment of cystinuria. Nat Rev Nephrol (2010) 1.40

Insulin stimulates long-chain fatty acid utilization by rat cardiac myocytes through cellular redistribution of FAT/CD36. Diabetes (2002) 1.38

Molecular basis of substrate-induced permeation by an amino acid antiporter. Proc Natl Acad Sci U S A (2011) 1.37

Identification of LAT4, a novel amino acid transporter with system L activity. J Biol Chem (2005) 1.36

The SLC3 and SLC7 families of amino acid transporters. Mol Aspects Med (2013) 1.36

Apolipoprotein C-III isofocusing in the diagnosis of genetic defects in O-glycan biosynthesis. Clin Chem (2003) 1.34

Role of mitochondrial dynamics proteins in the pathophysiology of obesity and type 2 diabetes. Int J Biochem Cell Biol (2009) 1.32

Caveolin-1 deficiency causes cholesterol-dependent mitochondrial dysfunction and apoptotic susceptibility. Curr Biol (2011) 1.31

PP2A regulatory subunit PP2A-B' counteracts S6K phosphorylation. Cell Metab (2010) 1.29

The role of viruses in the aetiology of community-acquired pneumonia in adults. Antivir Ther (2006) 1.28

Biophysical properties of mitochondrial fusion events in pancreatic beta-cells and cardiac cells unravel potential control mechanisms of its selectivity. Am J Physiol Cell Physiol (2010) 1.27

Caveolin-1 loss of function accelerates glucose transporter 4 and insulin receptor degradation in 3T3-L1 adipocytes. Endocrinology (2009) 1.26

Endoplasmic reticulum and the unfolded protein response: dynamics and metabolic integration. Int Rev Cell Mol Biol (2013) 1.23

The genetics of heteromeric amino acid transporters. Physiology (Bethesda) (2005) 1.18

Hormone-induced mitochondrial fission is utilized by brown adipocytes as an amplification pathway for energy expenditure. EMBO J (2014) 1.18

Localization and functional analyses of the MLC1 protein involved in megalencephalic leukoencephalopathy with subcortical cysts. Hum Mol Genet (2004) 1.18

A role for peroxisome proliferator-activated receptor γ coactivator-1 in the control of mitochondrial dynamics during postnatal cardiac growth. Circ Res (2013) 1.18

Arginine transport via cationic amino acid transporter 2 plays a critical regulatory role in classical or alternative activation of macrophages. J Immunol (2006) 1.15

Slc7a9-deficient mice develop cystinuria non-I and cystine urolithiasis. Hum Mol Genet (2003) 1.15

The nuclear cofactor DOR regulates autophagy in mammalian and Drosophila cells. EMBO Rep (2009) 1.14

Impact on fatty acid metabolism and differential localization of FATP1 and FAT/CD36 proteins delivered in cultured human muscle cells. Am J Physiol Cell Physiol (2005) 1.13

Cerebral folate deficiency and leukoencephalopathy caused by a mitochondrial DNA deletion. Ann Neurol (2006) 1.12

CD14 modulates inflammation-driven insulin resistance. Diabetes (2011) 1.11

Functional and structural characterization of the first prokaryotic member of the L-amino acid transporter (LAT) family: a model for APC transporters. J Biol Chem (2007) 1.11

rBAT-b(0,+)AT heterodimer is the main apical reabsorption system for cystine in the kidney. Am J Physiol Renal Physiol (2002) 1.11

Genetic and cellular studies of oxidative stress in methylmalonic aciduria (MMA) cobalamin deficiency type C (cblC) with homocystinuria (MMACHC). Hum Mutat (2009) 1.08

The role of amino acid transporters in inherited and acquired diseases. Biochem J (2011) 1.08

Coenzyme Q(10)-responsive ataxia: 2-year-treatment follow-up. Mov Disord (2010) 1.08

Identification of a novel modulator of thyroid hormone receptor-mediated action. PLoS One (2007) 1.08

Analysis of coenzyme Q10 in muscle and fibroblasts for the diagnosis of CoQ10 deficiency syndromes. Clin Biochem (2008) 1.07

Neuregulin signaling on glucose transport in muscle cells. J Biol Chem (2004) 1.07

[Human immunodeficiency virus-infected patients with community-acquired pneumonia: implication of respiratory viruses]. Enferm Infecc Microbiol Clin (2008) 1.06

Regulation of cardiac long-chain fatty acid and glucose uptake by translocation of substrate transporters. Pflugers Arch (2004) 1.05

Creatine transporter deficiency: prevalence among patients with mental retardation and pitfalls in metabolite screening. Clin Biochem (2007) 1.05

Two novel mutations in the BCKDK (branched-chain keto-acid dehydrogenase kinase) gene are responsible for a neurobehavioral deficit in two pediatric unrelated patients. Hum Mutat (2014) 1.01

Insulin stimulates mitochondrial fusion and function in cardiomyocytes via the Akt-mTOR-NFκB-Opa-1 signaling pathway. Diabetes (2013) 1.01

Exome sequencing identifies a new mutation in SERAC1 in a patient with 3-methylglutaconic aciduria. Mol Genet Metab (2013) 1.01

Membrane topology of system xc- light subunit reveals a re-entrant loop with substrate-restricted accessibility. J Biol Chem (2004) 1.01

Expression patterns of MLC1 protein in the central and peripheral nervous systems. Neurobiol Dis (2007) 1.00

A role for adipocyte-derived lipopolysaccharide-binding protein in inflammation- and obesity-associated adipose tissue dysfunction. Diabetologia (2013) 1.00

Alterations in the mitochondrial regulatory pathways constituted by the nuclear co-factors PGC-1alpha or PGC-1beta and mitofusin 2 in skeletal muscle in type 2 diabetes. Biochim Biophys Acta (2010) 1.00

Macrophages require distinct arginine catabolism and transport systems for proliferation and for activation. Eur J Immunol (2006) 1.00