The mitochondrial carrier SFXN1 is critical for complex III integrity and cellular metabolism

Defining the Substrate Spectrum of the TIM22 Complex Identifies Pyruvate Carrier Subunits as Unconventional Cargos

(2020) Ridhima Gomkale et al.   CURRENT BIOLOGY

Mitochondrial carrier protein overloading and misfolding induce aggresomes and proteostatic adaptations in the cytosol

(2019) Yaxin Liu et al.   MOLECULAR BIOLOGY OF THE CELL

Systematic Dissection of the Metabolic-Apoptotic Interface in AML Reveals Heme Biosynthesis to Be a Regulator of Drug Sensitivity

(2019) Kevin H. Lin et al.   Cell Metabolism

Sideroflexin 4 affects Fe-S cluster biogenesis, iron metabolism, mitochondrial respiration and heme biosynthetic enzymes

(2019) Bibbin T. Paul et al.   Scientific Reports

Glutamine via α-ketoglutarate dehydrogenase provides succinyl-CoA for heme synthesis during erythropoiesis

(2018) Joseph S. Burch et al.   BLOOD

Mitochondrial complex III Rieske Fe-S protein processing and assembly

(2018) Erika Fernandez-Vizarra et al.   CELL CYCLE

Rewiring of Glutamine Metabolism Is a Bioenergetic Adaptation of Human Cells with Mitochondrial DNA Mutations

(2018) Qiuying Chen et al.   Cell Metabolism

Serine Synthesis via PHGDH Is Essential for Heme Production in Endothelial Cells

(2018) Saar Vandekeere et al.   Cell Metabolism

Assembly of mammalian oxidative phosphorylation complexes I–V and supercomplexes

(2018) Alba Signes et al.   Essays in Biochemistry

Superoxide dismutases: Dual roles in controlling ROS damage and regulating ROS signaling

(2018) Ying Wang et al.   JOURNAL OF CELL BIOLOGY

Serine Catabolism by SHMT2 Is Required for Proper Mitochondrial Translation Initiation and Maintenance of Formylmethionyl-tRNAs

(2018) Denise R. Minton et al.   MOLECULAR CELL

The multifaceted contributions of mitochondria to cellular metabolism

(2018) Jessica B. Spinelli et al.   NATURE CELL BIOLOGY

Serine Availability Influences Mitochondrial Dynamics and Function through Lipid Metabolism

(2018) Xia Gao et al.   Cell Reports

Mutations in TIMM50 compromise cell survival in OxPhos‐dependent metabolic conditions

(2018) Aurelio Reyes et al.   EMBO Molecular Medicine

ACP Acylation Is an Acetyl-CoA-Dependent Modification Required for Electron Transport Chain Assembly

(2018) Jonathan G. Van Vranken et al.   MOLECULAR CELL

Mitochondrial translation requires folate-dependent tRNA methylation

(2018) Raphael J. Morscher et al.   NATURE

Regulation of mitochondrial iron homeostasis by sideroflexin 2

(2018) Ei Ei Mon et al.   Journal of Physiological Sciences

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

(2018) Nora Kory et al.   SCIENCE

Kaempferol increases levels of coenzyme Q in kidney cells and serves as a biosynthetic ring precursor

(2017) Lucía Fernández-del-Río et al.   FREE RADICAL BIOLOGY AND MEDICINE

The mammalian phosphate carrier SLC25A3 is a mitochondrial copper transporter required for cytochromecoxidase biogenesis

(2017) Aren Boulet et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Architecture Mapping of the Inner Mitochondrial Membrane Proteome by Chemical Tools in Live Cells

(2017) Song-Yi Lee et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

Human adenine nucleotide translocases physically and functionally interact with respirasomes

(2017) Ya-Wen Lu et al.   MOLECULAR BIOLOGY OF THE CELL

Sengers Syndrome-Associated Mitochondrial Acylglycerol Kinase Is a Subunit of the Human TIM22 Protein Import Complex

(2017) Yilin Kang et al.   MOLECULAR CELL

Acylglycerol Kinase Mutated in Sengers Syndrome Is a Subunit of the TIM22 Protein Translocase in Mitochondria

(2017) Milena Vukotic et al.   MOLECULAR CELL

TTC19 Plays a Husbandry Role on UQCRFS1 Turnover in the Biogenesis of Mitochondrial Respiratory Complex III

(2017) Emanuela Bottani et al.   MOLECULAR CELL

Functional Properties of the Mitochondrial Carrier System

(2017) Eric B. Taylor   TRENDS IN CELL BIOLOGY

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

Mitochondrial DNA Replication Defects Disturb Cellular dNTP Pools and Remodel One-Carbon Metabolism

(2016) Joni Nikkanen et al.   Cell Metabolism

TIM29 is a subunit of the human carrier translocase required for protein transport

(2016) Sylvie Callegari et al.   FEBS LETTERS

Defining functional classes of Barth syndrome mutation in humans

(2016) Ya-Wen Lu et al.   HUMAN MOLECULAR GENETICS

Novel Mechanisms for Heme-dependent Degradation of ALAS1 Protein as a Component of Negative Feedback Regulation of Heme Biosynthesis

(2016) Yoshiko Kubota et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The Plasma Membrane Protein Nce102 Implicated in Eisosome Formation Rescues a Heme Defect in Mitochondria

(2016) Hyung J. Kim et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The importance of serine metabolism in cancer

(2016) Katherine R. Mattaini et al.   JOURNAL OF CELL BIOLOGY

Metabolic regulation of mitochondrial dynamics

(2016) Prashant Mishra et al.   JOURNAL OF CELL BIOLOGY

The Mitochondrial Basis of Aging

(2016) Nuo Sun et al.   MOLECULAR CELL

APEX Fingerprinting Reveals the Subcellular Localization of Proteins of Interest

(2016) Song-Yi Lee et al.   Cell Reports

Tim29 is a novel subunit of the human TIM22 translocase and is involved in complex assembly and stability

(2016) Yilin Kang et al.   eLife

Mitochondrial dysfunction remodels one-carbon metabolism in human cells

(2016) Xiaoyan Robert Bao et al.   eLife

The mitochondrial phosphate carrier: Role in oxidative metabolism, calcium handling and mitochondrial disease

(2015) Erin L. Seifert et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Supporting Aspartate Biosynthesis Is an Essential Function of Respiration in Proliferating Cells

(2015) Lucas B. Sullivan et al.   CELL

MitoCarta2.0: an updated inventory of mammalian mitochondrial proteins

(2015) Sarah E. Calvo et al.   NUCLEIC ACIDS RESEARCH

Identification of the Mitochondrial Heme Metabolism Complex

(2015) Amy E. Medlock et al.   PLoS One

Nuclear gene mutations as the cause of mitochondrial complex III deficiency

(2015) Erika Fernández-Vizarra et al.   Frontiers in Genetics

Tracing Compartmentalized NADPH Metabolism in the Cytosol and Mitochondria of Mammalian Cells

(2014) Caroline A. Lewis et al.   MOLECULAR CELL

Serine and glycine metabolism in cancer

(2014) Ivano Amelio et al.   TRENDS IN BIOCHEMICAL SCIENCES

Activation of diverse signalling pathways by oncogenic PIK3CA mutations

(2014) Xinyan Wu et al.   Nature Communications

Serine, but Not Glycine, Supports One-Carbon Metabolism and Proliferation of Cancer Cells

(2014) Christiaan F. Labuschagne et al.   Cell Reports

Macrocytic Anemia and Mitochondriopathy Resulting from a Defect in Sideroflexin 4

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

Genome engineering using the CRISPR-Cas9 system

(2013) F Ann Ran et al.   Nature Protocols

RNA-Guided Human Genome Engineering via Cas9

(2013) P. Mali et al.   SCIENCE

LYRM7/MZM1L is a UQCRFS1 chaperone involved in the last steps of mitochondrial Complex III assembly in human cells

(2012) Ester Sánchez et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Structure, function, and assembly of heme centers in mitochondrial respiratory complexes

(2012) Hyung J. Kim et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Mitochondrial disorders as windows into an ancient organelle

(2012) Scott B. Vafai et al.   NATURE

Mechanisms of Protein Sorting in Mitochondria

(2012) D. Stojanovski et al.   Cold Spring Harbor Perspectives in Biology

Complete loss of expression of theANT1gene causing cardiomyopathy and myopathy

(2011) Andoni Echaniz-Laguna et al.   JOURNAL OF MEDICAL GENETICS

Metabolic control of mitochondrial biogenesis through the PGC-1 family regulatory network

(2010) Richard C. Scarpulla   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Molecular basis of hereditary iron homeostasis defects

(2010) Monika Horvathova et al.   Hematology

Hereditary sideroblastic anemia: pathophysiology and gene mutations

(2010) Hideo Harigae et al.   INTERNATIONAL JOURNAL OF HEMATOLOGY

Importing Mitochondrial Proteins: Machineries and Mechanisms

(2009) Agnieszka Chacinska et al.   CELL

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

Hemin promotes proliferation and differentiation of endothelial progenitor cells via activation of AKT and ERK

(2009) Jir-You Wang et al.   JOURNAL OF CELLULAR PHYSIOLOGY

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

(2009) Duane L Guernsey et al.   NATURE GENETICS

The ADP and ATP transport in mitochondria and its carrier

(2008) Martin Klingenberg   BIOCHIMICA ET BIOPHYSICA ACTA-BIOMEMBRANES

Cardiolipin defines the interactome of the major ADP/ATP carrier protein of the mitochondrial inner membrane

(2008) Steven M. Claypool et al.   JOURNAL OF CELL BIOLOGY