20,000 picometers under the OMM : diving into the vastness of mitochondrial metabolite transport

Systemic administration of AAV-Slc25a46 mitigates mitochondrial neuropathy in Slc25a46−/− mice

(2020) Li Yang et al.   HUMAN MOLECULAR GENETICS

The yeast mitochondrial pyruvate carrier is a hetero‐dimer in its functional state

(2019) Sotiria Tavoulari et al.   EMBO JOURNAL

Important Trends in UCP3 Investigation

(2019) Elena E. Pohl et al.   Frontiers in Physiology

The human uncoupling proteins 5 and 6 (UCP5/SLC25A14 and UCP6/SLC25A30) transport sulfur oxyanions, phosphate and dicarboxylates

(2019) Ruggiero Gorgoglione et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

MPC1 deletion is associated with poor prognosis and temozolomide resistance in glioblastoma

(2019) Yi Chai et al.   JOURNAL OF NEURO-ONCOLOGY

Sideroflexin 3 is a Mitochondrial Protein Enriched in Neurons

(2019) Aileen Rivell et al.   NEUROMOLECULAR MEDICINE

Impaired skeletal muscle mitochondrial pyruvate uptake rewires glucose metabolism to drive whole-body leanness

(2019) Arpit Sharma et al.   eLife

Identification of an ATP-sensitive potassium channel in mitochondria

(2019) Angela Paggio et al.   NATURE

BCAA catabolism in brown fat controls energy homeostasis through SLC25A44

(2019) Takeshi Yoneshiro et al.   NATURE

Disrupting Mitochondrial Pyruvate Uptake Directs Glutamine into the TCA Cycle away from Glutathione Synthesis and Impairs Hepatocellular Tumorigenesis

(2019) Sean C. Tompkins et al.   Cell Reports

The SLC25 Mitochondrial Carrier Family: Structure and Mechanism

(2019) Jonathan J. Ruprecht et al.   TRENDS IN BIOCHEMICAL SCIENCES

Regulation of Tumor Initiation by the Mitochondrial Pyruvate Carrier

(2019) Claire L. Bensard et al.   Cell Metabolism

A Variant of SLC1A5 Is a Mitochondrial Glutamine Transporter for Metabolic Reprogramming in Cancer Cells

(2019) Hee Chan Yoo et al.   Cell Metabolism

Expanding the phenotype of SLC25A42 -associated mitochondrial encephalomyopathy

(2018) M. Almannai et al.   CLINICAL GENETICS

The 2-oxoglutarate carrier promotes liver cancer by sustaining mitochondrial GSH despite cholesterol loading

(2018) Anna Baulies et al.   Redox Biology

Mitochondrial pyruvate carrier 1 functions as a tumor suppressor and predicts the prognosis of human renal cell carcinoma

(2018) Xiao-Peng Tang et al.   LABORATORY INVESTIGATION

SFXN1 is a mitochondrial serine transporter required for one-carbon metabolism

(2018) Nora Kory et al.   SCIENCE

An engineered thermal-shift screen reveals specific lipid preferences of eukaryotic and prokaryotic membrane proteins

(2018) Emmanuel Nji et al.   Nature Communications

MTCH2-mediated mitochondrial fusion drives exit from naïve pluripotency in embryonic stem cells

(2018) Amir Bahat et al.   Nature Communications

Screening of candidate substrates and coupling ions of transporters by thermostability shift assays

(2018) Homa Majd et al.   eLife

SLC25A10 biallelic mutations in intractable epileptic encephalopathy with complex I deficiency

(2017) Giuseppe Punzi et al.   HUMAN MOLECULAR GENETICS

Loss of SLC25A46 causes neurodegeneration by affecting mitochondrial dynamics and energy production in mice

(2017) Zhuo Li et al.   HUMAN MOLECULAR GENETICS

Reductions in the mitochondrial ABC transporter Abcb10 affect the transcriptional profile of heme biosynthesis genes

(2017) Alexandra Seguin et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Decreased Mitochondrial Pyruvate Transport Activity in the Diabetic Heart

(2017) Shraddha S. Vadvalkar et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Iron–sulfur cluster biogenesis and trafficking in mitochondria

(2017) Joseph J. Braymer et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Inhibition of the mitochondrial pyruvate carrier protects from excitotoxic neuronal death

(2017) Ajit S. Divakaruni et al.   JOURNAL OF CELL BIOLOGY

Rapid degradation of mutant SLC25A46 by the ubiquitin-proteasome system results in MFN1/2-mediated hyperfusion of mitochondria

(2017) Janos Steffen et al.   MOLECULAR BIOLOGY OF THE CELL

Functional Properties of the Mitochondrial Carrier System

(2017) Eric B. Taylor   TRENDS IN CELL BIOLOGY

Calcium regulation of the human mitochondrial ATP-Mg/Pi carrier SLC25A24 uses a locking pin mechanism

(2017) Steven P. D. Harborne et al.   Scientific Reports

Loss of forebrain MTCH2 decreases mitochondria motility and calcium handling and impairs hippocampal-dependent cognitive functions

(2017) Antonella Ruggiero et al.   Scientific Reports

Recurrent De Novo Dominant Mutations in SLC25A4 Cause Severe Early-Onset Mitochondrial Disease and Loss of Mitochondrial DNA Copy Number

(2016) Kyle Thompson et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Discoveries, metabolic roles and diseases of mitochondrial carriers: A review

(2016) Ferdinando Palmieri et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

SLC25A46is required for mitochondrial lipid homeostasis and cristae maintenance and is responsible for Leigh syndrome

(2016) Alexandre Janer et al.   EMBO Molecular Medicine

Shawn, the Drosophila Homolog of SLC25A39/40, Is a Mitochondrial Carrier That Promotes Neuronal Survival

(2016) J. R. Slabbaert et al.   JOURNAL OF NEUROSCIENCE

Uncoupling Protein, UCP-4 May Be Involved in Neuronal Defects During Aging and Resistance to Pathogens in Caenorhabditis elegans

(2016) Injeong Cho et al.   MOLECULES AND CELLS

The Warburg Effect: How Does it Benefit Cancer Cells?

(2016) Maria V. Liberti et al.   TRENDS IN BIOCHEMICAL SCIENCES

The severity of hereditary porphyria is modulated by the porphyrin exporter and Lan antigen ABCB6

(2016) Yu Fukuda et al.   Nature Communications

Sirt3 binds to and deacetylates mitochondrial pyruvate carrier 1 to enhance its activity

(2015) Lei Liang et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Hepatic Mitochondrial Pyruvate Carrier 1 Is Required for Efficient Regulation of Gluconeogenesis and Whole-Body Glucose Homeostasis

(2015) Lawrence R. Gray et al.   Cell Metabolism

Loss of Mitochondrial Pyruvate Carrier 2 in the Liver Leads to Defects in Gluconeogenesis and Compensation via Pyruvate-Alanine Cycling

(2015) Kyle S. McCommis et al.   Cell Metabolism

Glutathione-coordinated [2Fe–2S] cluster: a viable physiological substrate for mitochondrial ABCB7 transport

(2015) Jingwei Li et al.   CHEMICAL COMMUNICATIONS

Mutation of the mitochondrial carrier SLC25A42 causes a novel form of mitochondrial myopathy in humans

(2015) Hanan E. Shamseldin et al.   HUMAN GENETICS

Mutations in SLC25A46, encoding a UGO1-like protein, cause an optic atrophy spectrum disorder

(2015) Alexander J Abrams et al.   NATURE GENETICS

Proteome-wide drug and metabolite interaction mapping by thermal-stability profiling

(2015) Kilian V M Huber et al.   NATURE METHODS

Mitochondrial uncoupling links lipid catabolism to Akt inhibition and resistance to tumorigenesis

(2015) Sara M. Nowinski et al.   Nature Communications

A Role for the Mitochondrial Pyruvate Carrier as a Repressor of the Warburg Effect and Colon Cancer Cell Growth

(2014) John C. Schell et al.   MOLECULAR CELL

Glutamine Oxidation Maintains the TCA Cycle and Cell Survival during Impaired Mitochondrial Pyruvate Transport

(2014) Chendong Yang et al.   MOLECULAR CELL

Regulation of Substrate Utilization by the Mitochondrial Pyruvate Carrier

(2014) Nathaniel M. Vacanti et al.   MOLECULAR CELL

UCP2 transports C4 metabolites out of mitochondria, regulating glucose and glutamine oxidation

(2014) A. Vozza et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Structures of yeast mitochondrial ADP/ATP carriers support a domain-based alternating-access transport mechanism

(2014) J. J. Ruprecht et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Crystal Structures of Nucleotide-Free and Glutathione-Bound Mitochondrial ABC Transporter Atm1

(2014) V. Srinivasan et al.   SCIENCE

Calcium-induced conformational changes of the regulatory domain of human mitochondrial aspartate/glutamate carriers

(2014) Chancievan Thangaratnarajah et al.   Nature Communications

Macrocytic Anemia and Mitochondriopathy Resulting from a Defect in Sideroflexin 4

(2013) Gordon J. Hildick-Smith et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Joint Linkage and Association Analysis with Exome Sequence Data Implicates SLC25A40 in Hypertriglyceridemia

(2013) Elisabeth A. Rosenthal et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Mutations in ABCB6 Cause Dyschromatosis Universalis Hereditaria

(2013) Caie Zhang et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

Structures of ABCB10, a human ATP-binding cassette transporter in apo- and nucleotide-bound states

(2013) C. A. Shintre et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Missense mutations in the ABCB6 transporter cause dominant familialpseudohyperkalemia

(2012) Immacolata Andolfo et al.   AMERICAN JOURNAL OF HEMATOLOGY

Biogenesis of mitochondrial carrier proteins: Molecular mechanisms of import into mitochondria

(2012) Alessandra Ferramosca et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Mechanism of Fatty-Acid-Dependent UCP1 Uncoupling in Brown Fat Mitochondria

(2012) Andriy Fedorenko et al.   CELL

ATP-dependent Mitochondrial Porphyrin Importer ABCB6 Protects against Phenylhydrazine Toxicity

(2012) Dagny L. Ulrich et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Comprehensive molecular characterization of human colon and rectal cancer

(2012)   NATURE

ABCB6 is dispensable for erythropoiesis and specifies the new blood group system Langereis

(2012) Virginie Helias et al.   NATURE GENETICS

Shifting the Paradigm: The Putative Mitochondrial Protein ABCB6 Resides in the Lysosomes of Cells and in the Plasma Membrane of Erythrocytes

(2012) Katalin Kiss et al.   PLoS One

Disruption of ATP-binding cassette B8 in mice leads to cardiomyopathy through a decrease in mitochondrial iron export

(2012) Y. Ichikawa et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Identification and Functional Expression of the Mitochondrial Pyruvate Carrier

(2012) S. Herzig et al.   SCIENCE

A Mitochondrial Pyruvate Carrier Required for Pyruvate Uptake in Yeast, Drosophila, and Humans

(2012) D. K. Bricker et al.   SCIENCE

Structures of the nucleotide-binding domain of the human ABCB6 transporter and its complexes with nucleotides

(2010) Matthias Haffke et al.   ACTA CRYSTALLOGRAPHICA SECTION D-BIOLOGICAL CRYSTALLOGRAPHY

Discovery of Genes Essential for Heme Biosynthesis through Large-Scale Gene Expression Analysis

(2009) Roland Nilsson et al.   Cell Metabolism

A Novel Member of Solute Carrier Family 25 (SLC25A42) Is a Transporter of Coenzyme A and Adenosine 3′,5′-Diphosphate in Human Mitochondria

(2009) Giuseppe Fiermonte et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Pontocerebellar hypoplasia type III (CLAM): Extended phenotype and novel molecular findings

(2009) B. Durmaz et al.   JOURNAL OF NEUROLOGY

Mutations in mitochondrial carrier family gene SLC25A38 cause nonsyndromic autosomal recessive congenital sideroblastic anemia

(2009) Duane L Guernsey et al.   NATURE GENETICS

ABC transporters: the power to change

(2009) Douglas C. Rees et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

The Human and Mouse SLC25A29 Mitochondrial Transporters Rescue the Deficient Ornithine Metabolism in Fibroblasts of Patients With the Hyperornithinemia-Hyperammonemia-Homocitrullinuria (HHH) Syndrome

(2009) Josée A Camacho et al.   PEDIATRIC RESEARCH

Abcb10 physically interacts with mitoferrin-1 (Slc25a37) to enhance its stability and function in the erythroid mitochondria

(2009) W. Chen et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The evidence that the DNC (SLC25A19) is not the mitochondrial deoxyribonucleotide carrier

(2008) Jonghoon Kang et al.   MITOCHONDRION

The Role of the Iron Transporter ABCB7 in Refractory Anemia with Ring Sideroblasts

(2008) Jacqueline Boultwood et al.   PLoS One

A role for the mitochondrial deacetylase Sirt3 in regulating energy homeostasis

(2008) B.-H. Ahn et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The mechanism of transport by mitochondrial carriers based on analysis of symmetry

(2008) A. J. Robinson et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA