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Total 29 results found since Jan 2013.
Transcriptomic Profiling Identifies Ferroptosis-Related Gene Signatures in Ischemic Muscle Satellite Cells Affected by Peripheral Artery Disease
CONCLUSIONS: This is the first report documenting that genes known to be involved in ferroptosis are differentially expressed in human skeletal muscle affected by PAD. Targeting ferroptosis may be a novel therapeutic strategy to reduce PAD myopathy.PMID:37675635 | DOI:10.1161/ATVBAHA.123.319518
Source: Arteriosclerosis, Thrombosis and Vascular Biology - September 7, 2023 Category: Cardiology Authors: Lillian Tran Bowen Xie Edwyn Assaf Ricardo Ferrari Iraklis I Pipinos George P Casale Roberto Ivan Mota Alvidrez Simon Watkins Ulka Sachdev Source Type: research
Characteristics of the Isu1 C-terminus in relation to 2Fe-2S cluster assembly and ISCU Myopathy
In this report, the yeast ISCU ortholog "Isu1" has been characterized to gain a better understanding of the role of the scaffold protein in relation to [2Fe-2S] assembly and ISCU Myopathy. Here we explored the biophysical characteristics of the C-terminal region of Isu1, the segment of the protein that is truncated on the human ortholog during the disease ISCU Myopathy. We characterized the role of this region in relation to iron binding, protein stability, assembly of the ISC multiprotein complex required to accomplish Fe-S cluster assembly, and finally on overall cell viability. We determined the Isu1 C-terminus is essen...
Source: Journal of Biological Inorganic Chemistry : JBIC - October 30, 2022 Category: Chemistry Authors: Brianne E Lewis Courtney J Campbell Andria Rodrigues Lindsey Thompson Ashutosh K Pandey Sarah N Gallagher Debkumar Pain Andrew Dancis Timothy L Stemmler Source Type: research
Crohn & #39;s Disease Presenting as Metabolic Myopathy: A Case Report
We report a 20-yearr-old lady who presented with a painful proximal myopathy. On examination, she was malnourished with pallor, angular cheilitis, Bitots spots, and bilateral pitting pedal edema. Laboratory evaluation showed iron deficiency anemia, hypoalbuminemia, and very low vitamin D levels with elevated creatine phosphokinase levels. A possibility of osteomalacic metabolic myopathy due to vitamin D deficiency was considered. The malabsorption workup was negative. A colonoscopic biopsy showed noncaseating granulomatous inflammation suggestive of Crohn's disease. With supplementary therapy and specific treatm...
Source: Neurology India - July 1, 2022 Category: Neurology Authors: Sabu Kunnathuparambil George Neena Baby Prasanth Varghese Thalakottur Kuriakose Joseph Source Type: research
Skeletal Muscle MiR-210 Expression is Associated with Mitochondrial Function in Peripheral Artery Disease Patients
Transl Res. 2022 Mar 11:S1931-5244(22)00046-9. doi: 10.1016/j.trsl.2022.03.003. Online ahead of print.ABSTRACTPrevious studies have demonstrated that circulating microRNA (miR)-210 levels are elevated in peripheral artery disease (PAD) patients. MiR-210 is known to be a negative regulator of mitochondrial respiration; however, the relationship between miR-210 and mitochondrial function has yet to be studied in PAD. We aimed to compare skeletal muscle miR-210 expression of PAD patients to non-PAD controls (CON) and to examine the relationship between miR-210 expression and mitochondrial function. Skeletal muscle biopsies fr...
Source: Translational Research : the journal of laboratory and clinical medicine - March 15, 2022 Category: Laboratory Medicine Authors: Ahmed Ismaeel Emma Fletcher Dimitrios Miserlis Marissa Wechsler Evlampia Papoutsi Gleb Haynatzki Robert S Smith William T Bohannon Panagiotis Koutakis Source Type: research
The Influence of Cardiovascular Risk Factors and Hypogonadism on Cardiac Outcomes in an Aging Population of Beta-Thalassemia Patients
J Cardiovasc Dev Dis. 2021 Dec 21;9(1):3. doi: 10.3390/jcdd9010003.ABSTRACTBeta-thalassemia major (β-TM) is a hereditary genetic disease worsened by many comorbidities due to transfusion-related iron despite chelation therapy. Since there has recently been an increase in life expectancy of patients to up to 50 years old, which influences the prevalence of these diseases and the time span for traditional cardiovascular risk factors to play their role, this study aims to evaluate their distribution and prevalence in a population of thalassemia major patients and their relationship with observed cardiovascular events and pot...
Source: Atherosclerosis - January 20, 2022 Category: Cardiology Authors: Umberto Barbero Matteo Ajassa Carmen Maria Gaglioti Antonio Piga Giovanni Battista Ferrero Filomena Longo Source Type: research
Augmenter of Liver Regeneration regulates cellular iron homeostatis by modulating mitochondrial transport of ATP-binding cassette B8
Chronic loss of Augmenter of Liver Regeneration (ALR) results in mitochondrial myopathy with cataracts, however, the mechanism for this disorder remains unclear. Here, we demonstrate that loss of ALR, a principal component of the MIA40/ALR protein import pathway, results in impaired cytosolic Fe/S cluster biogenesis in mammalian cells. Mechanistically, MIA40/ALR facilitates the mitochondrial import of ATP binding cassette (ABC)-B8, an inner mitochondrial membrane protein required for cytoplasmic Fe/S cluster maturation, through physical interaction with ABCB8. Downregulation of ALR impairs mitochondrial ABCB8 import, reduc...
Source: eLife - April 9, 2021 Category: Biomedical Science Tags: Cell Biology Medicine Source Type: research
Augmenter of liver regeneration regulates cellular iron homeostasis by modulating mitochondrial transport of ATP-binding cassette B8
Chronic loss of Augmenter of Liver Regeneration (ALR) results in mitochondrial myopathy with cataracts; however, the mechanism for this disorder remains unclear. Here, we demonstrate that loss of ALR, a principal component of the MIA40/ALR protein import pathway, results in impaired cytosolic Fe/S cluster biogenesis in mammalian cells. Mechanistically, MIA40/ALR facilitates the mitochondrial import of ATP-binding cassette (ABC)-B8, an inner mitochondrial membrane protein required for cytoplasmic Fe/S cluster maturation, through physical interaction with ABCB8. Downregulation of ALR impairs mitochondrial ABCB8 import, reduc...
Source: eLife - April 9, 2021 Category: Biomedical Science Tags: Cell Biology Medicine Source Type: research
