The metabolic and lipidomic profiling of the effects of tracheal occlusion in a rabbit model of congenital diaphragmatic hernia

Easton, Zachary J W; Regnault, Timothy R. H.; Mudri, Martina; Zhao, Shuang; Buzatto, Adriana Zardini; Li, Janet; Duruisseau-Kuntz, Ryan; Davidson, Jacob; Li, Liang; Bütter, Andreana

doi:10.1016/j.jpedsurg.2023.01.040

Cited by 2 publications

(1 citation statement)

References 37 publications

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“…MS/MS spectra were acquired for sample alignment, and identification was processed by NovaMT LipidScreener. MS/MS peaks were identified by a three-tier identification approach based on MS/MS spectral similarity, retention time, and accurate mass match (Tier 1: MS/MS match score ≥500, precursor m/z error ≤20.0 ppm, 5.0 mDa; Tier 2: MS/MS match score <500; precursor m/z error ≤20.0 ppm, 5.0 mDa; Tier 3: Mass match with m/z error ≤20.0 ppm and 5.0 mDa) (Easton et al, 2023). All compounds identified in these tiers were utilized for normalization and data analysis.…”

Section: Methodsmentioning

confidence: 99%

The ER Thioredoxin-Related Transmembrane Protein TMX2 Controls Redox-Mediated Tethering of ER-Mitochondria Contacts (ERMCS)

Chen,

Yap,

Bassot

et al. 2024

Preprint

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Thioredoxin-related transmembrane proteins (TMX) of the endoplasmic reticulum (ER) have emerged as key regulators of ER membrane properties. Within the ER lumen, TMX proteins and other ER redox enzymes determine oxidative conditions, which control the formation of ER-mitochondria membrane contacts (ERMCS) and determine their function. ERMCS exhibit cytoplasmic redox nanodomains, derived from ER and mitochondrial reactive oxygen species (ROS), whose mechanistic regulation is uncharacterized. Our research has identified the ER protein TMX2, which uses its unique cytosolic thioredoxin domain to prevent cytosolic sulfenylation of mitochondrial outer membrane proteins such as TOM70 through a functional interaction with peroxiredoxin-1 (PRDX1). By doing so, TMX2 interferes with the TOM70 ERMCS tethering function and reduces mitochondrial Ca2+ flux and metabolism. Recently, TMX2 mutations have been identified to cause a neurodevelopmental disorder with microcephaly, cortical malformations, and spasticity (NEDMCMS). Using TMX2-mutated NEDMCMS patient cells, we demonstrate that compromising TMX2 through mutation reproduces mitochondrial defects. In a fly in vivo model, TMX2 knockdown manifests predominantly in glial cells. Our results therefore provide important mechanistic insight into NEDMCMS and mechanistically link TMX2-mediated control of ERMCS to brain development and function.

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Section: Methodsmentioning

confidence: 99%

The ER Thioredoxin-Related Transmembrane Protein TMX2 Controls Redox-Mediated Tethering of ER-Mitochondria Contacts (ERMCS)

Chen,

Yap,

Bassot

et al. 2024

Preprint

Self Cite

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Lipidomic Approaches in Common and Rare Cerebrovascular Diseases: The Discovery of Unconventional Lipids as Novel Biomarkers

Potenza

Gorla

Carrozzini

et al. 2023

IJMS

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Stroke remains a major cause of death and disability worldwide. Identifying new circulating biomarkers able to distinguish and monitor common and rare cerebrovascular diseases that lead to stroke is of great importance. Biomarkers provide complementary information that may improve diagnosis, prognosis and prediction of progression as well. Furthermore, biomarkers can contribute to filling the gap in knowledge concerning the underlying disease mechanisms by pointing out novel potential therapeutic targets for personalized medicine. If many “conventional” lipid biomarkers are already known to exert a relevant role in cerebrovascular diseases, the aim of our study is to review novel “unconventional” lipid biomarkers that have been recently identified in common and rare cerebrovascular disorders using novel, cutting-edge lipidomic approaches.

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The metabolic and lipidomic profiling of the effects of tracheal occlusion in a rabbit model of congenital diaphragmatic hernia

Cited by 2 publications

References 37 publications

The ER Thioredoxin-Related Transmembrane Protein TMX2 Controls Redox-Mediated Tethering of ER-Mitochondria Contacts (ERMCS)

The ER Thioredoxin-Related Transmembrane Protein TMX2 Controls Redox-Mediated Tethering of ER-Mitochondria Contacts (ERMCS)

Lipidomic Approaches in Common and Rare Cerebrovascular Diseases: The Discovery of Unconventional Lipids as Novel Biomarkers

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