A mitochondrial SCF‐FBXL4 ubiquitin E3 ligase complex degrades BNIP3 and NIX to restrain mitophagy and prevent mitochondrial disease

Dichloroacetate improves mitochondrial function, physiology, and morphology in FBXL4 disease models

(2022) Manuela Lavorato et al.   JCI Insight

UBXD8 mediates mitochondria‐associated degradation to restrain apoptosis and mitophagy

(2022) Jing Zheng et al.   EMBO REPORTS

MTCH2 is a mitochondrial outer membrane protein insertase

(2022) Alina Guna et al.   SCIENCE

Mitochondrial cristae architecture protects against mtDNA release and inflammation

(2022) Baiyu He et al.   Cell Reports

Molecular mechanisms and physiological functions of mitophagy

(2021) Mashun Onishi et al.   EMBO JOURNAL

Mitophagy receptor FUNDC1 is regulated by PGC‐1α/NRF1 to fine tune mitochondrial homeostasis

(2021) Lei Liu et al.   EMBO REPORTS

BNIP3 promotes HIF‐1α‐driven melanoma growth by curbing intracellular iron homeostasis

(2021) Mónica Vara‐Pérez et al.   EMBO JOURNAL

Glycerol-3-phosphate biosynthesis regenerates cytosolic NAD+ to alleviate mitochondrial disease

(2021) Shanshan Liu et al.   Cell Metabolism

Highly accurate protein structure prediction with AlphaFold

(2021) John Jumper et al.   NATURE

FBXL 4 deficiency increases mitochondrial removal by autophagy

(2020) David Alsina et al.   EMBO Molecular Medicine

Mitochondrial Quality Control and Restraining Innate Immunity

(2020) Andrew T. Moehlman et al.   Annual Review of Cell and Developmental Biology

Basal Mitophagy Occurs Independently of PINK1 in Mouse Tissues of High Metabolic Demand

(2018) Thomas G. McWilliams et al.   Cell Metabolism

Atlastins remodel the endoplasmic reticulum for selective autophagy

(2018) Jin Rui Liang et al.   JOURNAL OF CELL BIOLOGY

Molecular and clinical spectra of FBXL4 deficiency

(2017) Ayman W. El-Hattab et al.   HUMAN MUTATION

BCL-2 family proteins as regulators of mitochondria metabolism

(2016) Atan Gross   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

A novel mutation in FBXL4 in a Norwegian child with encephalomyopathic mitochondrial DNA depletion syndrome 13

(2016) Tuva Barøy et al.   European Journal of Medical Genetics

mito-QC illuminates mitophagy and mitochondrial architecture in vivo

(2016) Thomas G. McWilliams et al.   JOURNAL OF CELL BIOLOGY

Urolithin A induces mitophagy and prolongs lifespan in C. elegans and increases muscle function in rodents

(2016) Dongryeol Ryu et al.   NATURE MEDICINE

Clinical, morphological, biochemical, imaging and outcome parameters in 21 individuals with mitochondrial maintenance defect related to FBXL4 mutations

(2015) Martina Huemer et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Measuring In Vivo Mitophagy

(2015) Nuo Sun et al.   MOLECULAR CELL

Coordination of mitophagy and mitochondrial biogenesis during ageing in C. elegans

(2015) Konstantinos Palikaras et al.   NATURE

The Roles of PINK1, Parkin, and Mitochondrial Fidelity in Parkinson’s Disease

(2015) Alicia M. Pickrell et al.   NEURON

CRISPR-Cas9 Knockin Mice for Genome Editing and Cancer Modeling

(2014) Randall J. Platt et al.   CELL

Mutations in FBXL4 Cause Mitochondrial Encephalopathy and a Disorder of Mitochondrial DNA Maintenance

(2013) Penelope E. Bonnen et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Mutations in FBXL4, Encoding a Mitochondrial Protein, Cause Early-Onset Mitochondrial Encephalomyopathy

(2013) Xiaowu Gai et al.   AMERICAN JOURNAL OF HUMAN GENETICS

A Neo-Substrate that Amplifies Catalytic Activity of Parkinson’s-Disease-Related Kinase PINK1

(2013) Nicholas T. Hertz et al.   CELL

Loss of iron triggers PINK1/Parkin-independent mitophagy

(2013) George F G Allen et al.   EMBO REPORTS

Novel Small-Molecule PGC-1  Transcriptional Regulator With Beneficial Effects on Diabetic db/db Mice

(2012) L.-N. Zhang et al.   DIABETES

Mitochondrial outer-membrane protein FUNDC1 mediates hypoxia-induced mitophagy in mammalian cells

(2012) Lei Liu et al.   NATURE CELL BIOLOGY

Autophagy: Renovation of Cells and Tissues

(2011) Noboru Mizushima et al.   CELL

A Sensitive and Quantitative Technique for Detecting Autophagic Events Based on Lysosomal Delivery

(2011) Hiroyuki Katayama et al.   CHEMISTRY & BIOLOGY

Mechanisms of mitophagy

(2010) Richard J. Youle et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

PINK1 Is Selectively Stabilized on Impaired Mitochondria to Activate Parkin

(2010) Derek P. Narendra et al.   PLOS BIOLOGY

Role of BNIP3 and NIX in cell death, autophagy and mitophagy

(2009) J Zhang et al.   CELL DEATH AND DIFFERENTIATION

Hypoxia-Induced Autophagy Is Mediated through Hypoxia-Inducible Factor Induction of BNIP3 and BNIP3L via Their BH3 Domains

(2009) G. Bellot et al.   MOLECULAR AND CELLULAR BIOLOGY

Mitochondrial Autophagy Is an HIF-1-dependent Adaptive Metabolic Response to Hypoxia

(2008) Huafeng Zhang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Parkin is recruited selectively to impaired mitochondria and promotes their autophagy

(2008) Derek Narendra et al.   JOURNAL OF CELL BIOLOGY

Essential role for Nix in autophagic maturation of erythroid cells

(2008) Hector Sandoval et al.   NATURE