Mechanisms of mitochondrial iron-sulfur protein biogenesis
R Lill, SA Freibert - Annual review of biochemistry, 2020 - annualreviews.org
Mitochondria are essential in most eukaryotes and are involved in numerous biological
functions including ATP production, cofactor biosyntheses, apoptosis, lipid synthesis, and …
functions including ATP production, cofactor biosyntheses, apoptosis, lipid synthesis, and …
Mammalian iron–sulfur cluster biogenesis: Recent insights into the roles of frataxin, acyl carrier protein and ATPase-mediated transfer to recipient proteins
N Maio, A Jain, TA Rouault - Current opinion in chemical biology, 2020 - Elsevier
The recently solved crystal structures of the human cysteine desulfurase NFS1, in complex
with the LYR protein ISD11, the acyl carrier protein ACP, and the main scaffold ISCU, have …
with the LYR protein ISD11, the acyl carrier protein ACP, and the main scaffold ISCU, have …
Mitochondrial [4Fe-4S] protein assembly involves reductive [2Fe-2S] cluster fusion on ISCA1–ISCA2 by electron flow from ferredoxin FDX2
BD Weiler, MC Brück, I Kothe, E Bill… - Proceedings of the …, 2020 - National Acad Sciences
The essential process of iron-sulfur (Fe/S) cluster assembly (ISC) in mitochondria occurs in
three major phases. First,[2Fe-2S] clusters are synthesized on the scaffold protein ISCU2; …
three major phases. First,[2Fe-2S] clusters are synthesized on the scaffold protein ISCU2; …
Outlining the complex pathway of mammalian Fe-S cluster biogenesis
N Maio, TA Rouault - Trends in biochemical sciences, 2020 - cell.com
Iron–sulfur (Fe-S) clusters (ISCs) are ubiquitous cofactors essential to numerous
fundamental cellular processes. Assembly of ISCs and their insertion into apoproteins …
fundamental cellular processes. Assembly of ISCs and their insertion into apoproteins …
Molecular and genetic analysis of resistance to METI-I acaricides in Iranian populations of the citrus red mite Panonychus citri
ES Alavijeh, J Khajehali, S Snoeck, R Panteleri… - Pesticide biochemistry …, 2020 - Elsevier
The citrus red mite, Panonychus citri, is a major pest on citrus all around the world.
Mitochondrial Electron Transport Inhibitors of complex I (METI-I) acaricides such as …
Mitochondrial Electron Transport Inhibitors of complex I (METI-I) acaricides such as …
Update review about metabolic myopathies
J Finsterer - Life, 2020 - mdpi.com
The aim of this review is to summarize and discuss recent findings and new insights in the
etiology and phenotype of metabolic myopathies. The review relies on a systematic literature …
etiology and phenotype of metabolic myopathies. The review relies on a systematic literature …
[HTML][HTML] ISCU interacts with NFU1, and ISCU [4Fe-4S] transfers its Fe-S cluster to NFU1 leading to the production of holo-NFU1
K Cai, RO Frederick, JL Markley - Journal of structural biology, 2020 - Elsevier
NFU1 is a late-acting factor in the biogenesis of human mitochondrial iron-sulfur proteins.
Mutations in NFU1 are associated with genetic diseases such as multiple mitochondrial …
Mutations in NFU1 are associated with genetic diseases such as multiple mitochondrial …
Pyrostigmine therapy in a patient with VAMP1-related congenital myasthenic syndrome
MA Al-Muhaizea, L AlQuait, A AlRasheed… - Neuromuscular …, 2020 - Elsevier
Congenital myasthenic syndrome comprises several genetic disorders that impair
neuromuscular junction transmission. Causative mutations occur in at least 30 genes …
neuromuscular junction transmission. Causative mutations occur in at least 30 genes …
Ancient founder mutation in RUBCN: a second unrelated family confirms Salih ataxia (SCAR15)
MZ Seidahmed, MH Hamad, A AlBakheet, SA Elmalik… - BMC neurology, 2020 - Springer
Background Homozygous frameshift mutation in RUBCN (KIAA0226), known to result in
endolysosomal machinery defects, has previously been reported in a single Saudi family …
endolysosomal machinery defects, has previously been reported in a single Saudi family …
Expanding the genotype-phenotype spectrum of ISCA2-related multiple mitochondrial dysfunction syndrome-cavitating leukoencephalopathy and prolonged survival
TG Hartman, K Yosovich, HG Michaeli, L Blumkin… - neurogenetics, 2020 - Springer
Iron-sulfur cluster assembly 2 (ISCA2)-related multiple mitochondrial dysfunction syndrome
4 (MMDS4) is a fatal autosomal recessive mitochondrial leukoencephalopathy. The disease …
4 (MMDS4) is a fatal autosomal recessive mitochondrial leukoencephalopathy. The disease …