Mitochondrial dysfunction in the pathophysiology of renal diseases

R Che, Y Yuan, S Huang… - American Journal of …, 2014 - journals.physiology.org
Mitochondrial dysfunction has gained recognition as a contributing factor in many diseases.
The kidney is a kind of organ with high energy demand, rich in mitochondria. As such …

[HTML][HTML] p53 and mitochondrial function in neurons

DB Wang, C Kinoshita, Y Kinoshita… - Biochimica et Biophysica …, 2014 - Elsevier
The p53 tumor suppressor plays a central role in dictating cell survival and death as a
cellular sensor for a myriad of stresses including DNA damage, oxidative and nutritional …

Tumor suppressor p53 and its gain-of-function mutants in cancer

J Liu, C Zhang, Z Feng - Acta Biochim Biophys Sin, 2014 - academic.oup.com
Tumor suppressor p53 plays a pivotal role in tumor suppression. p53 is the most frequently
mutated gene in cancer. As a transcription factor, p53 mainly exerts its role in tumor …

CD147 promotes reprogramming of glucose metabolism and cell proliferation in HCC cells by inhibiting the p53-dependent signaling pathway

Q Huang, J Li, J Xing, W Li, H Li, X Ke, J Zhang… - Journal of …, 2014 - Elsevier
Background & Aims Cancer cells exhibit the reprogrammed metabolism characterized by
high level of glycolysis even in the presence of oxygen. Aerobic glycolysis, known as the …

[HTML][HTML] Thioredoxin and glutaredoxin-mediated redox regulation of ribonucleotide reductase

R Sengupta, A Holmgren - World journal of biological chemistry, 2014 - ncbi.nlm.nih.gov
Ribonucleotide reductase (RNR), the rate-limiting enzyme in DNA synthesis, catalyzes
reduction of the different ribonucleotides to their corresponding deoxyribonucleotides. The …

Deoxypyrimidine monophosphate bypass therapy for thymidine kinase 2 deficiency

C Garone, B Garcia‐Diaz, V Emmanuele… - EMBO molecular …, 2014 - embopress.org
Autosomal recessive mutations in the thymidine kinase 2 gene (TK2) cause mitochondrial
DNA depletion, multiple deletions, or both due to loss of TK 2 enzyme activity and ensuing …

Administration of deoxyribonucleosides or inhibition of their catabolism as a pharmacological approach for mitochondrial DNA depletion syndrome

Y Cámara, E González-Vioque… - Human molecular …, 2014 - academic.oup.com
Mitochondrial DNA (mtDNA) depletion syndrome (MDS) is characterized by a reduction in
mtDNA copy number and consequent mitochondrial dysfunction in affected tissues. A …

Linear mtDNA fragments and unusual mtDNA rearrangements associated with pathological deficiency of MGME1 exonuclease

TJ Nicholls, G Zsurka, V Peeva, S Schöler… - Human molecular …, 2014 - academic.oup.com
MGME1, also known as Ddk1 or C20orf72, is a mitochondrial exonuclease found to be
involved in the processing of mitochondrial DNA (mtDNA) during replication. Here, we …

[HTML][HTML] Mitochondrial genome changes and neurodegenerative diseases

M Pinto, CT Moraes - Biochimica et Biophysica Acta (BBA)-Molecular Basis …, 2014 - Elsevier
Mitochondria are essential organelles within the cell where most of the energy production
occurs by the oxidative phosphorylation system (OXPHOS). Critical components of the …

Cellular regulation of ribonucleotide reductase in eukaryotes

E Guarino, I Salguero, SE Kearsey - Seminars in cell & developmental …, 2014 - Elsevier
Synthesis of deoxynucleoside triphosphates (dNTPs) is essential for both DNA replication
and repair and a key step in this process is catalyzed by ribonucleotide reductases (RNRs) …