Results 31 to 40 of about 5,673 (157)

Prolonged treatment with pimelic o-aminobenzamide HDAC inhibitors ameliorates the disease phenotype of a Friedreich ataxia mouse model [PDF]

, 2011
NOTICE: this is the author’s version of a work that was accepted for publication in Neurobiology of Disease. Changes resulting from the publishing process, such as peer review, editing, corrections, structural formatting, and other quality control ...
Ezzatizadeh, V, Rusche, James R., Steve Jones, James R. Rusche, Gottesfeld, Joel M., Jones, S, Sandi, C, Barnes, Glenn, Joel M. Gottesfeld, Pook, MA, Pinto, RM, Al-Mahdawi, Sahar, Chiranjeevi Sandi, Sandi, Chiranjeevi, Barnes, G, Mark A. Pook, Pinto, Ricardo Mouro, Al-Mahdawi, S, Jones, Steve, Pook, Mark A., Vahid Ezzatizadeh, Ezzatizadeh, Vahid, Sahar Al-Mahdawi, Rusche, JR, Ricardo Mouro Pinto, Gottesfeld, JM, Glenn Barnes   +26 more
core   +1 more source

Ferrochelatase activity of plant frataxin [PDF]

Biochimie, 2019
Frataxin plays a key role in cellular iron homeostasis of different organisms. It is engaged in several activities at the FeS cluster assembly machinery and it is also involved in heme biosynthesis. In plants, two genes encoding ferrochelatases (FC1 and FC2) catalyze the incorporation of iron into protoporphyrin IX in the last stage of heme synthesis ...
Alejandro M. Armas, Manuel Balparda, Agustina Terenzi, Maria V. Busi, Maria A. Pagani, Diego F. Gomez-Casati   +5 more
openaire   +3 more sources

The Ferroxidase Activity of Yeast Frataxin [PDF]

Journal of Biological Chemistry, 2002
Frataxin is required for maintenance of normal mitochondrial iron levels and respiration. The mature form of yeast frataxin (mYfh1p) assembles stepwise into a multimer of 840 kDa (alpha(48)) that accumulates iron in a water-soluble form. Here, two distinct iron oxidation reactions are shown to take place during the initial assembly step (alpha ...
Sungjo, Park, Oleksandr, Gakh, Steven M, Mooney, Grazia, Isaya   +3 more
openaire   +2 more sources

The Friedreich ataxia GAA repeat expansion mutation induces comparable epigenetic changes in human and transgenic mouse brain and heart tissues [PDF]

, 2007
Friedreich ataxia (FRDA) is caused by a homozygous GAA repeat expansion mutation within intron 1 of the FXN gene, leading to reduced expression of frataxin protein.
Pook, MA, Mouro Pinto, R, Al-Mahdawi, S, Trabzuni, D, Sandi, C, S. Al-Mahdawi, D. Trabzuni, S. Lymperi, D. Varshney, O. Ismail, C. Sandi, R. M. Pinto, M. Pook   +12 more
core   +2 more sources

Identification of Frataxin as a regulator of ferroptosis

Redox Biology, 2020
Ferroptosis is a newly discovered form of non-apoptotic regulated cell death and is characterized by iron-dependent and lipid peroxidation. Due to the enhanced dependence on iron in cancer cells, induction of ferroptosis is becoming a promising therapeutic strategy.
Jing Du, Yi Zhou, Yanchun Li, Jun Xia, Yongjian Chen, Sufeng Chen, Xin Wang, Weidong Sun, Tongtong Wang, Xueying Ren, Xu Wang, Yihan An, Kang Lu, Wanye Hu, Siyuan Huang, Jianghui Li, Xiangmin Tong, Ying Wang   +17 more
openaire   +3 more sources

Crystal Structure of Human Frataxin [PDF]

Journal of Biological Chemistry, 2000
Friedreich's ataxia, an autosomal recessive neurodegenerative disorder characterized by progressive gait and limb ataxia, cardiomyopathy, and diabetes mellitus, is caused by decreased frataxin production or function. The structure of human frataxin, which we have determined at 1.8-A resolution, reveals a novel protein fold.
S, Dhe-Paganon, R, Shigeta, Y I, Chi, M, Ristow, S E, Shoelson   +4 more
openaire   +2 more sources

Posttranslational regulation of mitochondrial frataxin and identification of compounds that increase frataxin levels in Friedreich’s ataxia

Journal of Biological Chemistry, 2022
Friedreich's ataxia (FRDA) is a degenerative disease caused by a decrease in the mitochondrial protein frataxin (Fxn), which is involved in iron-sulfur cluster (ISC) synthesis. Diminutions in Fxn result in decreased ISC synthesis, increased mitochondrial iron accumulation, and impaired mitochondrial function.
Hackett, Peter T., Jia, Xuan, Li, Liangtao, Ward, Diane M.   +3 more
openaire   +2 more sources

Magnetoreceptor Promotes Hepatocellular Carcinoma Development and Blunts Sorafenib‐Induced Ferroptosis by Regulating Iron Homeostasis

Advanced Science, EarlyView.
Magnetoreceptor (MagR) regulates iron homeostasis and mitochondrial function in hepatocellular carcinoma (HCC) cells. Specifically, MagR deficiency suppresses tumor cell proliferation, migration, and invasion and enhances sorafenib‐induced ferroptosis through iron accumulation.
Tao Wang, Chenghua Song, Xin He, Mengzhou Wang, Wuming Liu, Xiaolin Wang, Yuanyuan Zhang, Jia Zhang, Lin Zhang, Dan Li, Yu Zhang, Zheng Wu, Yi Lyu, Rongqian Wu   +13 more
wiley   +1 more source

Key Interventions in Friedreich's Ataxia and Their Impact on Patient Outcomes: A Systematic Review

Movement Disorders, EarlyView.
Abstract Friedreich's ataxia (FA) is a rare neurodegenerative disease with multisystemic symptoms that requires multidisciplinary care. This systematic review summarizes available pharmacological and nonpharmacological interventions, their outcomes, and alignment with patient‐centered care domains, as well as their impact on these domains.
Dorota Sarwinska, Maresa Buchholz, Audrey Iskandar, Marcus Grobe‐Einsler, Thomas Klockgether, Jennifer Faber, Bernhard Michalowsky   +6 more
wiley   +1 more source

Directional information flow in human frataxin defines allosteric pathways connecting the hydrophobic core to the iron‐binding ridge

The FEBS Journal, EarlyView.
Human frataxin deficiency causes Friedreich's ataxia, yet how iron‐binding events are communicated across the protein is unclear. Using transfer entropy analysis of molecular dynamics simulations, we identify buried hydrophobic core leucines (LEU136, LEU140) as the source of directional signaling toward the iron‐binding acidic ridge.
Kevser Kübra Kırboğa, Ecir Uğur Küçüksille   +1 more
wiley   +1 more source