Protein damage, repair and proteolysis

N Chondrogianni, I Petropoulos, S Grimm… - Molecular aspects of …, 2014 - Elsevier
Proteins are continuously affected by various intrinsic and extrinsic factors. Damaged
proteins influence several intracellular pathways and result in different disorders and …

[HTML][HTML] Mitochondrial quality control and communications with the nucleus are important in maintaining mitochondrial function and cell health

VN Kotiadis, MR Duchen, LD Osellame - Biochimica et Biophysica Acta …, 2014 - Elsevier
Background The maintenance of cell metabolism and homeostasis is a fundamental
characteristic of living organisms. In eukaryotes, mitochondria are the cornerstone of these …

RNA sequencing identifies dysregulation of the human pancreatic islet transcriptome by the saturated fatty acid palmitate

M Cnop, B Abdulkarim, G Bottu, DA Cunha… - Diabetes, 2014 - Am Diabetes Assoc
Pancreatic β-cell dysfunction and death are central in the pathogenesis of type 2 diabetes
(T2D). Saturated fatty acids cause β-cell failure and contribute to diabetes development in …

[HTML][HTML] ATP-dependent Lon protease controls tumor bioenergetics by reprogramming mitochondrial activity

PM Quirós, Y Espanol, R Acín-Pérez, F Rodríguez… - Cell reports, 2014 - cell.com
We generated mice deficient in Lon protease (LONP1), a major enzyme of the mitochondrial
quality control machinery. Homozygous deletion of Lonp1 causes early embryonic lethality …

[HTML][HTML] PINK1-Parkin pathway activity is regulated by degradation of PINK1 in the mitochondrial matrix

RE Thomas, LA Andrews, JL Burman, WY Lin… - PLoS …, 2014 - journals.plos.org
Loss-of-function mutations in PINK1, which encodes a mitochondrially targeted
serine/threonine kinase, result in an early-onset heritable form of Parkinson's disease …

[HTML][HTML] The ER mitochondria calcium cycle and ER stress response as therapeutic targets in amyotrophic lateral sclerosis

V Tadic, T Prell, J Lautenschlaeger… - Frontiers in cellular …, 2014 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by
progressive loss of upper and lower motor neurons. Although the etiology remains unclear …

Mitochondrial matrix proteases as novel therapeutic targets in malignancy

CA Goard, AD Schimmer - Oncogene, 2014 - nature.com
Although mitochondrial function is often altered in cancer, it remains essential for tumor
viability. Tight control of protein homeostasis is required for the maintenance of …

Mitochondrial ATP-dependent proteases in protection against accumulation of carbonylated proteins

E Smakowska, M Czarna, H Janska - Mitochondrion, 2014 - Elsevier
Carbonylation is an irreversible oxidative modification of proteins induced by reactive
oxygen species (ROS) and reactive nitrogen species (RNS) or by-products of oxidative …

[HTML][HTML] Inhibition of Lon blocks cell proliferation, enhances chemosensitivity by promoting apoptosis and decreases cellular bioenergetics of bladder cancer: potential …

Y Liu, L Lan, K Huang, R Wang, C Xu, Y Shi, X Wu… - Oncotarget, 2014 - ncbi.nlm.nih.gov
ATP-dependent Lon protease within mitochondrial matrix contributes to the degradation of
abnormal proteins. The oxidative or hypoxic stress which represents the stress phenotype of …

Control of mitochondrial integrity in ageing and disease

R Szklarczyk, M Nooteboom… - … Transactions of the …, 2014 - royalsocietypublishing.org
Various molecular and cellular pathways are active in eukaryotes to control the quality and
integrity of mitochondria. These pathways are involved in keeping a 'healthy'population of …