Janne Purhonen

Hepatic gene replacement restores energy metabolism and doubles the survival in mouse model of GRACILE syndrome, a neonatal mitochondrial disease

This study uses gene replacement in a mouse model of GRACILE syndrome to show improved energy metabolism and doubled survival through liver-specific correction of a mitochondrial complex III deficiency.

ISG15-USP18 signaling restrains viperin-dependent metabolic antiviral restriction

This study shows that ISG15-USP18 signaling limits viperin-dependent metabolic antiviral restriction by stabilizing USP18 and reducing viperin expression, thereby inhibiting viral replication.

Evolutionarily conserved temperature dependency leads to loss of protein O-GlcNAc in mammalian hypothermia

This study identifies temperature as an evolutionarily conserved regulator of O-GlcNAcylation, showing that hypothermia reduces O-GlcNAc levels across species.

Hepatic mitochondrial respiration is crucial for euthermia in complex III-deficient mice with impaired brown adipose tissue thermogenesis

This study shows that restoring mitochondrial complex III function in the liver of CIII-deficient mice prevents hypothermia and improves survival by enabling normal thermoregulation.

Quantification of all 12 canonical ribonucleotides by real-time fluorogenic in vitro transcription

The paper develops a chromatography-free assay to quantify all 12 canonical ribonucleotides in biological samples using real-time fluorogenic detection during in vitro transcription.

A sensitive assay for dNTPs based on long synthetic oligonucleotides, EvaGreen dye, and inhibitor-resistant high-fidelity DNA polymerase

The paper introduces a sensitive microplate assay for quantifying dNTPs using long synthetic oligonucleotides, EvaGreen dye, and inhibitor-resistant DNA polymerase, enabling reliable measurement in mouse tissues.