Cur@ SF NPs alleviate Friedreich's ataxia in a mouse model through synergistic iron chelation and antioxidation

L Xu, Z Sun, Z Xing, Y Liu, H Zhao, Z Tang… - Journal of …, 2022 - Springer
Abnormal iron metabolism, mitochondrial dysfunction and the derived oxidative damage are
the main pathogeneses of Friedrich's ataxia (FRDA), a single-gene inherited recessive …

The Regulation of the Disease-Causing Gene FXN

YN Dong, E Mercado-Ayón, J Coulman, L Flatley… - Cells, 2024 - mdpi.com
Friedreich's ataxia (FRDA) is a progressive neurodegenerative disease caused in almost all
patients by expanded guanine–adenine–adenine (GAA) trinucleotide repeats within intron 1 …

[HTML][HTML] Steroidogenic electron-transfer factors and their diseases

WL Miller - Annals of Pediatric Endocrinology & Metabolism, 2021 - ncbi.nlm.nih.gov
Most steroidogenesis disorders are caused by mutations in genes encoding the
steroidogenic enzymes, but work in the past 20 years has identified related disorders …

ATP-Binding Cassette Transporter of Clinical Significance: Sideroblastic Anemia

JO Ogunbileje, N Harris, T Wynn, R Kashif… - Journal of Personalized …, 2024 - mdpi.com
The ATP-binding cassette (ABC) transporters are a vast group of 48 membrane proteins,
some of which are of notable physiological and clinical importance. Some ABC transporters …

Functionalization of mesoporous silica with a GA-Mismatched dsDNA chain for efficient identification and selective capturing of the MutY protein

J Sun, J Wang, X Chen - ACS Applied Materials & Interfaces, 2023 - ACS Publications
MUTYH adenine DNA glycosylase and its homologous protein (collectively MutY) are typical
DNA glycosylases with a [4Fe4S] cluster and a helix–hairpin–helix (HhH) motif in its …

Mechanistic insights into tRNA thiolation catalyzed by iron-sulfur enzymes

石坂優人 - 2023 - eprints.lib.hokudai.ac.jp
tRNA transfers amino acids to ribosomes to translate genetic information into proteins.
However, immature tRNA does not function immediately after transcription. Hence, tRNA …

[HTML][HTML] ミトコンドリア代謝を知るためのメタボロミクス

瀬戸山大樹 - Journal of Japanese Biochemical Society, 2022 - seikagaku.jbsoc.or.jp
細胞小器官の一つであるミトコンドリアは,「細胞のエネルギー発電所 (powerhouse)」 と呼ばれる.
もちろん, エネルギーとは ATP のことであり,「発電所」 とは内膜に存在する酸化的リン酸化システム …