Results 51 to 60 of about 899,069 (291)

Mitochondrial diseases

Biochimica et Biophysica Acta (BBA) - Bioenergetics, 2004
By convention, the term "mitochondrial diseases" refers to disorders of the mitochondrial respiratory chain, which is the only metabolic pathway in the cell that is under the dual control of the mitochondrial genome (mtDNA) and the nuclear genome (nDNA).
openaire   +2 more sources

Organ‐specific redox imbalances in spinal muscular atrophy mice are partially rescued by SMN antisense oligonucleotides

FEBS Letters, EarlyView.
We identified a systemic, progressive loss of protein S‐glutathionylation—detected by nonreducing western blotting—alongside dysregulation of glutathione‐cycle enzymes in both neuronal and peripheral tissues of Taiwanese SMA mice. These alterations were partially rescued by SMN antisense oligonucleotide therapy, revealing persistent redox imbalance as ...
Sofia Vrettou, Brunhilde Wirth
wiley   +1 more source

Primary mitochondrial disease in the US: Data from patients and physicians' perspective on health care delivery

Data in Brief, 2019
This article presents data that examine the patient's perception of health care delivery for mitochondrial disease in the US. It also presents the opinions of mitochondrial disease expert physicians about creating a specialised network of clinics to ...
Amel Karaa, Amy Goldstein, Cristy Balcells, Kira Mann, Laura Stanley, Philip E. Yeske, Sumit Parikh   +6 more
doaj   +1 more source

Transferrin receptor 1‐mediated iron uptake supports thermogenic activation in human cervical‐derived adipocytes

FEBS Letters, EarlyView.
In this study, we found that human cervical‐derived adipocytes maintain intracellular iron level by regulating the expression of iron transport‐related proteins during adrenergic stimulation. Melanotransferrin is predicted to interact with transferrin receptor 1 based on in silico analysis.
Rahaf Alrifai, Mizuki Seo, Gyath Karadsheh, Fachrur Rizal Mahendra, Máté Á Demény, Ferenc Győry, János András Mótyán, László Fésüs, Endre Kristóf, Rini Arianti   +9 more
wiley   +1 more source

The epidemiology and mutation types of Leber’s hereditary optic neuropathy in Thailand

Annals of Medicine, 2022
Purpose Leber’s hereditary optic neuropathy (LHON), the most common mitochondrial optic neuropathy, causes visual loss, especially in young adults. Due to the absence of epidemiological data in Southeast Asia, we aimed to determine Thai LHON patients ...
Kanchalika Sathianvichitr, Benjaporn Sigkaman, Niphon Chirapapaisan, Poramaet Laowanapiban, Tanyatuth Padungkiatsagul, Supanut Apinyawasisuk, Juthamat Witthayaweerasak, Wanicha Chuenkongkaew   +7 more
doaj   +1 more source

Altered brain energetics induces mitochondrial fission arrest in Alzheimer's Disease. [PDF]

, 2016
Altered brain metabolism is associated with progression of Alzheimer's Disease (AD). Mitochondria respond to bioenergetic changes by continuous fission and fusion.
Bachmeier, Benjamin V, Christensen, Trace A, Diaz Brinton, Roberta, Gateno, Benjamin, Gylys, Karen H, Itoh, Kie, Parisi, Joseph E, Patterson, Emily R, Poon, Wayne W, Salisbury, Jeffrey L, Schroeder, Andreas, Sesaki, Hiromi, Trushin, Sergey, Trushina, Eugenia, Yao, Jia, Zhang, Liang   +15 more
core   +1 more source

Gut microbiome and aging—A dynamic interplay of microbes, metabolites, and the immune system

FEBS Letters, EarlyView.
Age‐dependent shifts in microbial communities engender shifts in microbial metabolite profiles. These in turn drive shifts in barrier surface permeability of the gut and brain and induce immune activation. When paired with preexisting age‐related chronic inflammation this increases the risk of neuroinflammation and neurodegenerative diseases.
Aaron Mehl, Eran Blacher
wiley   +1 more source

The combined effects of physical exercise training and detraining on adiponectin in overweight and obese children

Integrative Medicine Research, 2013
Background: The purpose of this study is to assess the combined exercise programs (12 weeks' physical exercise training, resistance and aerobic) and 6 weeks' detraining on the correlation of metabolic syndrome (MS) markers and plasma adiponectin level in
Jae-Young Jeon, Jin Han, Hyun-Jun Kim, Moon Soo Park, Dae Yun Seo, Yi-Sub Kwak   +5 more
doaj   +1 more source

Linking neurogenesis, oligodendrogenesis, and myelination defects to neurodevelopmental disruption in primary mitochondrial disorders

FEBS Letters, EarlyView.
Mitochondrial remodeling shapes neural and glial lineage progression by matching metabolic supply with demand. Elevated OXPHOS supports differentiation and myelin formation, while myelin compaction lowers mitochondrial dependence, revealing mitochondria as key drivers of developmental energy adaptation.
Sahitya Ranjan Biswas, Porter L. Tomsick, Alicia M. Pickrell, Paul D. Morton   +3 more
wiley   +1 more source

A gated hydrophobic funnel within BAX binds bioactive lipids to potentiate pro-apoptotic function

Nature Communications
Mitochondria maintain a distinct biochemical environment that cooperates with pro-apoptotic BAX and BH3‑only proteins (e.g., BIM) to promote mitochondrial outer membrane permeabilization (MOMP), the key event to initiate physiological and pharmacological
Jesse D. Gelles, Yiyang Chen, Mark P. A. Luna-Vargas, Ariele Viacava Follis, Md Abdullah Al Noman, Md Kabir, Stella G. Bayiokos, Jarvier N. Mohammed, Tara M. Sebastian, Ngoc Dung Pham, Yi Shi, Jian Jin, Richard W. Kriwacki, Jerry Edward Chipuk   +13 more
doaj   +1 more source