N-glycanase NGLY1 regulates mitochondrial homeostasis and inflammation through NRF1

Transcriptome and functional analysis in a Drosophila model of NGLY1 deficiency provides insight into therapeutic approaches

(2018) Katie G Owings et al.   HUMAN MOLECULAR GENETICS

Mitochondrial function requires NGLY1

(2018) Jianping Kong et al.   MITOCHONDRION

Brown adipose tissue thermogenic adaptation requires Nrf1-mediated proteasomal activity

(2018) Alexander Bartelt et al.   NATURE MEDICINE

Immune Diseases Associated with TREX1 and STING Dysfunction

(2017) Nan Yan   JOURNAL OF INTERFERON AND CYTOKINE RESEARCH

Lethality of mice bearing a knockout of the Ngly1-gene is partially rescued by the additional deletion of the Engase gene

(2017) Haruhiko Fujihira et al.   PLoS Genetics

Inhibition of NGLY1 Inactivates the Transcription Factor Nrf1 and Potentiates Proteasome Inhibitor Cytotoxicity

(2017) Frederick M. Tomlin et al.   ACS Central Science

Dengue virus NS2B protein targets cGAS for degradation and prevents mitochondrial DNA sensing during infection

(2017) Sebastian Aguirre et al.   Nature Microbiology

Transcription factor NFE2L2/NRF2 is a regulator of macroautophagy genes

(2016) Marta Pajares et al.   Autophagy

Fanconi Anemia Proteins Function in Mitophagy and Immunity

(2016) Rhea Sumpter et al.   CELL

The cytoplasmic peptide: N -glycanase (NGLY1) — Structure, expression and cellular functions

(2016) Tadashi Suzuki et al.   GENE

Prospective phenotyping of NGLY1-CDDG, the first congenital disorder of deglycosylation

(2016) Christina Lam et al.   GENETICS IN MEDICINE

Type I interferon–mediated monogenic autoinflammation: The type I interferonopathies, a conceptual overview

(2016) Mathieu P. Rodero et al.   JOURNAL OF EXPERIMENTAL MEDICINE

RNase H2 catalytic core Aicardi-Goutières syndrome–related mutant invokes cGAS–STING innate immune-sensing pathway in mice

(2016) Vladislav Pokatayev et al.   JOURNAL OF EXPERIMENTAL MEDICINE

RPA and Rad51 constitute a cell intrinsic mechanism to protect the cytosol from self DNA

(2016) Christine Wolf et al.   Nature Communications

A congenital disorder of deglycosylation: Biochemical characterization ofN-glycanase 1 deficiency in patient fibroblasts

(2015) Ping He et al.   GLYCOBIOLOGY

DNA Damage Primes the Type I Interferon System via the Cytosolic DNA Sensor STING to Promote Anti-Microbial Innate Immunity

(2015) Anetta Härtlova et al.   IMMUNITY

Cutting Edge: Inhibiting TBK1 by Compound II Ameliorates Autoimmune Disease in Mice

(2015) Maroof Hasan et al.   JOURNAL OF IMMUNOLOGY

Mitochondrial DNA stress primes the antiviral innate immune response

(2015) A. Phillip West et al.   NATURE

Endo-β-N-acetylglucosaminidase formsN-GlcNAc protein aggregates during ER-associated degradation in Ngly1-defective cells

(2015) Chengcheng Huang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Innate Immune Sensing and Signaling of Cytosolic Nucleic Acids

(2014) Jiaxi Wu et al.   Annual Review of Immunology

Apoptotic Caspases Suppress mtDNA-Induced STING-Mediated Type I IFN Production

(2014) Michael J. White et al.   CELL

Apoptotic Caspases Prevent the Induction of Type I Interferons by Mitochondrial DNA

(2014) Anthony Rongvaux et al.   CELL

PMI: A ΔΨm Independent Pharmacological Regulator of Mitophagy

(2014) Daniel A. East et al.   CHEMISTRY & BIOLOGY

Mutations in NGLY1 cause an inherited disorder of the endoplasmic reticulum–associated degradation pathway

(2014) Gregory M. Enns et al.   GENETICS IN MEDICINE

Integrated transcriptome analysis across mitochondrial disease etiologies and tissues improves understanding of common cellular adaptations to respiratory chain dysfunction

(2014) Zhe Zhang et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Intrinsic Self-DNA Triggers Inflammatory Disease Dependent on STING

(2014) Jeonghyun Ahn et al.   JOURNAL OF IMMUNOLOGY

Improved vectors and genome-wide libraries for CRISPR screening

(2014) Neville E Sanjana et al.   NATURE METHODS

Nrf2 and Nrf1 signaling and ER stress crosstalk: implication for proteasomal degradation and autophagy

(2013) Hadi Digaleh et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Autoimmunity Initiates in Nonhematopoietic Cells and Progresses via Lymphocytes in an Interferon-Dependent Autoimmune Disease

(2012) Alevtina Gall et al.   IMMUNITY

Trex1 regulates lysosomal biogenesis and interferon-independent activation of antiviral genes

(2012) Maroof Hasan et al.   NATURE IMMUNOLOGY

Atomic structures of the eukaryotic ribosome

(2012) Sebastian Klinge et al.   TRENDS IN BIOCHEMICAL SCIENCES

Central nervous system-specific deletion of transcription factor Nrf1 causes progressive motor neuronal dysfunction

(2011) Akira Kobayashi et al.   GENES TO CELLS

New insights into the role of mitochondria in aging: mitochondrial dynamics and more

(2010) A. Y. Seo et al.   JOURNAL OF CELL SCIENCE

Transcription Factor Nrf1 Mediates the Proteasome Recovery Pathway after Proteasome Inhibition in Mammalian Cells

(2010) Senthil K. Radhakrishnan et al.   MOLECULAR CELL

SLP-2 is required for stress-induced mitochondrial hyperfusion

(2009) Daniel Tondera et al.   EMBO JOURNAL

Trex1 Prevents Cell-Intrinsic Initiation of Autoimmunity

(2008) Daniel B. Stetson et al.   CELL