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Article
Open AccessFabry Disease: Cardiac Implications and Molecular Mechanisms
This review explores the interplay among metabolic dysfunction, oxidative stress, inflammation, and fibrosis in Fabry disease, focusing on their potential implications for cardiac involvement. We aim to discus...
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Article
Open AccessActivation of the integrated stress response rewires cardiac metabolism in Barth syndrome
Barth Syndrome (BTHS) is an inherited cardiomyopathy caused by defects in the mitochondrial transacylase TAFAZZIN (Taz), required for the synthesis of the phospholipid cardiolipin. BTHS is characterized by heart ...
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Article
Open AccessMitochondrial dysfunction promotes the transition of precursor to terminally exhausted T cells through HIF-1α-mediated glycolytic reprogramming
T cell exhaustion is a hallmark of cancer and persistent infections, marked by inhibitory receptor upregulation, diminished cytokine secretion, and impaired cytolytic activity. Terminally exhausted T cells are...
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Article
Open AccessCardiac Involvement in Mitochondrial Disorders
We review pathophysiology and clinical features of mitochondrial disorders manifesting with cardiomyopathy.
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Article
Open AccessCooperative STAT/NF-κB signaling regulates lymphoma metabolic reprogramming and aberrant GOT2 expression
Knowledge of stromal factors that have a role in the transcriptional regulation of metabolic pathways aside from c-Myc is fundamental to improvements in lymphoma therapy. Using a MYC-inducible human B-cell line, ...
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Article
Motor-free mitochondrial presequence translocase drives membrane integration of preproteins
The mitochondrial inner membrane is the central energy-converting membrane of eukaryotic cells. The electrochemical proton gradient generated by the respiratory chain drives the ATP synthase. To maintain this ...
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Article
Pam16 has an essential role in the mitochondrial protein import motor
Mitochondrial preproteins destined for the matrix are translocated by two channel-forming transport machineries, the translocase of the outer membrane and the presequence translocase of the inner membrane. The...
