(Q37763723)

22 August 2017

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title

Roles of mitochondria in human disease (English)

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22 August 2017

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1

1 reference

22 August 2017

Gyorgy Szabadkai

2

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22 August 2017

1 January 2010

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22 August 2017

Essays in Biochemistry

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22 August 2017

47

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22 August 2017

115-137

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INTRAMITOCHONDRIAL FIBERS WITH DNA CHARACTERISTICS. I. FIXATION AND ELECTRON STAINING REACTIONS

22 August 2017

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

The pathophysiology of mitochondrial disease as modeled in the mouse

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Transcriptional paradigms in mammalian mitochondrial biogenesis and function

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Mitochondrial DNA replication and repair: all a flap

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

The organization and inheritance of the mitochondrial genome

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Inherited mitochondrial diseases of DNA replication

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

A mitochondrial paradigm of metabolic and degenerative diseases, aging, and cancer: a dawn for evolutionary medicine

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Disorders from perturbations of nuclear-mitochondrial intergenomic cross-talk.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Inherited mitochondrial optic neuropathies

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Mitochondrial ND5 gene variation associated with encephalomyopathy and mitochondrial ATP consumption

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

A mitochondrial protein compendium elucidates complex I disease biology

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Building the mitochondrial proteome

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Integrated analysis of protein composition, tissue diversity, and gene regulation in mouse mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Mitochondrial dynamics in mammalian health and disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Mitochondrial dynamics and apoptosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Quality control of mitochondria: protection against neurodegeneration and ageing

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Mitochondrial-nuclear communications

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Proteotoxic stress and inducible chaperone networks in neurodegenerative disease and aging

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Protein quality control in mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Ubiquitin-dependent and -independent mitochondrial protein quality controls: implications in ageing and neurodegenerative diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Lon protease preferentially degrades oxidized mitochondrial aconitase by an ATP-stimulated mechanism

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Downregulation of the human Lon protease impairs mitochondrial structure and function and causes cell death

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Translating m-AAA protease function in mitochondria to hereditary spastic paraplegia

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Molecular basis of inherited spastic paraplegias

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Traffic accidents: molecular genetic insights into the pathogenesis of the hereditary spastic paraplegias

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Hereditary spastic paraplegia SPG13 is associated with a mutation in the gene encoding the mitochondrial chaperonin Hsp60

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Translocation of proteins into mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

The life span determinant p66Shc localizes to mitochondria where it associates with mitochondrial heat shock protein 70 and regulates trans-membrane potential.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Involvement of mortalin in cellular senescence from the perspective of its mitochondrial import, chaperone, and oxidative stress management functions

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Glucose regulated proteins in cancer progression, drug resistance and immunotherapy

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

From proliferative to neurological role of an hsp70 stress chaperone, mortalin

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Functions and dysfunctions of mitochondrial dynamics

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Mitochondrial 'kiss-and-run': interplay between mitochondrial motility and fusion-fission dynamics

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Impaired autophagy: A mechanism of mitochondrial dysfunction in anoxic rat hepatocytes

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Mitochondrial fusion, fission and autophagy as a quality control axis: the bioenergetic view

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Regulation of mitochondrial fusion and division

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

The machines that divide and fuse mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Mitochondrial fusion in human cells is efficient, requires the inner membrane potential, and is mediated by mitofusins.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Mitochondrial fusion protects against neurodegeneration in the cerebellum.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Mutations in the mitochondrial GTPase mitofusin 2 cause Charcot-Marie-Tooth neuropathy type 2A

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Autophagy: molecular machinery for self-eating

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Uth1p is involved in the autophagic degradation of mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Aup1p, a yeast mitochondrial protein phosphatase homolog, is required for efficient stationary phase mitophagy and cell survival

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

IF1, the endogenous regulator of the F(1)F(o)-ATPsynthase, defines mitochondrial volume fraction in HeLa cells by regulating autophagy

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

ROS, mitochondria and the regulation of autophagy

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Reactive oxygen species are essential for autophagy and specifically regulate the activity of Atg4

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Endophilin B1 is required for the maintenance of mitochondrial morphology

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Bif-1 interacts with Beclin 1 through UVRAG and regulates autophagy and tumorigenesis

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Down-regulation of Bax-interacting factor-1 in colorectal adenocarcinoma

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

The pathogenesis of Friedreich ataxia and the structure and function of frataxin

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Beta-amyloid fragment 25-35 causes mitochondrial dysfunction in primary cortical neurons

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Accumulation of amyloid precursor protein in the mitochondrial import channels of human Alzheimer's disease brain is associated with mitochondrial dysfunction.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Expression of mutant SOD1 in astrocytes induces functional deficits in motoneuron mitochondria

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

A cell biological perspective on mitochondrial dysfunction in Parkinson disease and other neurodegenerative diseases

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Loss of Omi mitochondrial protease activity causes the neuromuscular disorder of mnd2 mutant mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Neuroprotective role of the Reaper-related serine protease HtrA2/Omi revealed by targeted deletion in mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

The mitochondrial protease HtrA2 is regulated by Parkinson's disease-associated kinase PINK1.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

PINK1 protects against oxidative stress by phosphorylating mitochondrial chaperone TRAP1

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

PINK1-associated Parkinson's disease is caused by neuronal vulnerability to calcium-induced cell death

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Impaired mitochondrial dynamics and function in the pathogenesis of Parkinson's disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Premature ageing in mice expressing defective mitochondrial DNA polymerase

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Mitochondrial DNA mutations, oxidative stress, and apoptosis in mammalian aging

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Does premature aging of the mtDNA mutator mouse prove that mtDNA mutations are involved in natural aging?

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

The p66shc adaptor protein controls oxidative stress response and life span in mammals

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Ca2+ -dependent interaction of S100A1 with F1-ATPase leads to an increased ATP content in cardiomyocytes

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Characterization of SCaMC-3-like/slc25a41, a novel calcium-independent mitochondrial ATP-Mg/Pi carrier.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Recombinant expression of the Ca(2+)-sensitive aspartate/glutamate carrier increases mitochondrial ATP production in agonist-stimulated Chinese hamster ovary cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Nitric oxide, cytochrome C oxidase, and the cellular response to hypoxia.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Mitochondrial NO and reactive nitrogen species production: does mtNOS exist?

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Reactive oxygen species in cancer cells: live by the sword, die by the sword.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Mitochondrial intermembrane junctional complexes and their involvement in cell death

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Mitochondrial control of cell death

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Voltage-dependent anion channels are dispensable for mitochondrial-dependent cell death

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

The ADP/ATP translocator is not essential for the mitochondrial permeability transition pore

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Loss of cyclophilin D reveals a critical role for mitochondrial permeability transition in cell death

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Properties of the permeability transition pore in mitochondria devoid of Cyclophilin D.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Cyclophilin D inactivation protects axons in experimental autoimmune encephalomyelitis, an animal model of multiple sclerosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Reconstituted adenine nucleotide translocase forms a channel for small molecules comparable to the mitochondrial permeability transition pore

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Not all mitochondrial carrier proteins support permeability transition pore formation: no involvement of uncoupling protein 1.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Transient and long-lasting openings of the mitochondrial permeability transition pore can be monitored directly in intact cells by changes in mitochondrial calcein fluorescence

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

The cyclophilin inhibitor Debio 025 normalizes mitochondrial function, muscle apoptosis and ultrastructural defects in Col6a1-/- myopathic mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Quercetin can act either as an inhibitor or an inducer of the mitochondrial permeability transition pore: A demonstration of the ambivalent redox character of polyphenols.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

On the involvement of a cyclosporin A sensitive mitochondrial pore in myocardial reperfusion injury

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

The role of the mitochondrial permeability transition pore in heart disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Cyclophilin D is a component of mitochondrial permeability transition and mediates neuronal cell death after focal cerebral ischemia

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Mechanisms underlying the loss of mitochondrial membrane potential in glutamate excitotoxicity

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Mitochondrial injury in pancreatitis

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Mitochondrial dysfunction and apoptosis in myopathic mice with collagen VI deficiency.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Cyclosporine A treatment for Ullrich congenital muscular dystrophy: a cellular study of mitochondrial dysfunction and its rescue

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

On the pathogenesis of collagen VI muscular dystrophies--comment on article of Hicks et al.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Genetic ablation of cyclophilin D rescues mitochondrial defects and prevents muscle apoptosis in collagen VI myopathic mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Cyclosporin A corrects mitochondrial dysfunction and muscle apoptosis in patients with collagen VI myopathies

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

The effect of nitric oxide on cell respiration: A key to understanding its role in cell survival or death.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Regulation of mitochondrial structure and function by the F1Fo-ATPase inhibitor protein, IF1.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Mitochondria and Ca(2+)in cell physiology and pathophysiology

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Mitochondrial modulation of oxygen-dependent radiosensitivity in some human tumour cell lines

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Pharmacological profile of the selective mitochondrial F1F0 ATP hydrolase inhibitor BMS-199264 in myocardial ischemia

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

IF(1): setting the pace of the F(1)F(o)-ATP synthase

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

21 January 2018

The Hallmarks of Cancer

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Understanding the Warburg effect: the metabolic requirements of cell proliferation

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

PET/CT in radiation oncology

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Tumor suppressors and cell metabolism: a recipe for cancer growth

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Tumor cell metabolism: cancer's Achilles' heel

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Regulation of tumor pH and the role of carbonic anhydrase 9.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Implications of mitochondrial DNA mutations and mitochondrial dysfunction in tumorigenesis

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Mitochondria in cancer cells: what is so special about them?

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Cytoplasmic functions of the tumour suppressor p53.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

The biology of cancer: metabolic reprogramming fuels cell growth and proliferation

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

New roles for pyruvate kinase M2: working out the Warburg effect

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

A mitochondria-K+ channel axis is suppressed in cancer and its normalization promotes apoptosis and inhibits cancer growth

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Mitochondrial DNA mutations in cancer: a review.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

ROS-generating mitochondrial DNA mutations can regulate tumor cell metastasis

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Metabolic transformation in cancer.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Transformation of human mesenchymal stem cells increases their dependency on oxidative phosphorylation for energy production

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Mitochondria as therapeutic targets for cancer chemotherapy

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Dichloroacetate (DCA) as a potential metabolic-targeting therapy for cancer

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Impaired PGC-1alpha function in muscle in Huntington's disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Mitochondrial gene therapy augments mitochondrial physiology in a Parkinson's disease cell model

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Glucose metabolism and programmed cell death: an evolutionary and mechanistic perspective

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Mutations in the mitochondrial genome confer resistance of cancer cells to anticancer drugs.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

No death without life: vital functions of apoptotic effectors

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Mitochondria as targets for chemotherapy

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Mitochondria mediated cell death in diabetes

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

beta-Cell death during progression to diabetes

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Apoptosis in the pathophysiology of diabetes mellitus

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Mechanisms of disease:Molecular and metabolic mechanisms of insulin resistance and beta-cell failure in type 2 diabetes

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Impact of mitochondrial calcium on the coupling of metabolism to insulin secretion in the pancreatic beta-cell

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

In beta-cells, mitochondria integrate and generate metabolic signals controlling insulin secretion

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

The cytokine interleukin-1beta reduces the docking and fusion of insulin granules in pancreatic beta-cells, preferentially decreasing the first phase of exocytosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Cell death and endoplasmic reticulum stress: disease relevance and therapeutic opportunities

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Interleukin 1 beta induces the formation of nitric oxide by beta-cells purified from rodent islets of Langerhans. Evidence for the beta-cell as a source and site of action of nitric oxide

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Inhibition of AMP-activated protein kinase protects pancreatic beta-cells from cytokine-mediated apoptosis and CD8+ T-cell-induced cytotoxicity.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Poly (ADP-ribose) polymerase inhibition prevents spontaneous and recurrent autoimmune diabetes in NOD mice by inducing apoptosis of islet-infiltrating leukocytes

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

The role of peroxisome proliferator-activated receptor gamma coactivator-1 beta in the pathogenesis of fructose-induced insulin resistance.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Glucolipotoxicity: fuel excess and beta-cell dysfunction.

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

Metabolic coupling factors in pancreatic beta-cell signal transduction

21 January 2018

1 reference

https://api.crossref.org/works/10.1042%2FBSE0470115

21 January 2018

Identifiers

DOI

10.1042/BSE0470115

1 reference

22 August 2017

1 reference

PubMed ID

20533904