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Clinical Manifestation of Mitochondrial Disorders in Childhood
Published in Shamim I. Ahmad, Handbook of Mitochondrial Dysfunction, 2019
Leigh syndrome (Leigh, 1951) itself has two different meanings. The first represents the radiological or pathological findings of focal bilaterally symmetrical lesions, especially in the thalamus and brainstem regions. The other broadens this meaning to the clinical unit also known as subacute necrotizing encephalomyelopathy. Genetically, LS is very heterogenous and should be defined in by specific mutation or protein deficit where possible, as some particular may specifically differ in their clinical manifestation (e.g., SURF1 or pyruvate-dehydrogenase complex deficiency). In general, LS may be caused by deficits of respiratory chain complex subunits (complex I, II, IV, and V) and their cofactors (e.g., co-enzyme Q10), mutations in nDNA (e.g., SCO2, SURF1), mtDNA encoded tRNA, or the pyruvate dehydrogenase complex (Loeffen et al., 2000; Finsterer, 2008). Mitochondrial respiratory chain complex I (nicotinamide adenine dinucleotide-ubiquinone oxidoreductase) is the largest enzymatic complex of the mitochondrial respiratory chain. Defects in complex I due to nuclear DNA mutations are one of the most frequent casuses of LS. Various mutations in subunits of complex I encoded by nDNA (NDUFV1, NDUFV2, NDUFS1, NDUFS2, NDUFS3, NDUFS4, NDUFS7, and NDUFS8 were reported (Marin et al., 2013).
Effects of psychoactive drugs on cellular bioenergetic pathways
Published in The World Journal of Biological Psychiatry, 2021
Chiara C. Bortolasci, Briana Spolding, Srisaiyini Kidnapillai, Mark F. Richardson, Nina Vasilijevic, Sheree D. Martin, Laura J. Gray, Sean L. McGee, Michael Berk, Ken Walder
After genome-wide correction for multiple testing using FDR, valproate significantly increased the expression of NDUFA13 (q = 0.037), NDUFA3 (q = 0.00012), NDUFB3 (q = 0.011), NDUFS8 (q = 0.049), NDUFV1 (q = 0.0090) and NDUFV3 (q = 0.00015) from Complex 1, UQCRFS1 (q = 0.030) and UQCRH (q = 3.6E-06) from Complex 3, COX4l1 (q = 0.038), COX6A1 (q = 0.036), COX7A2L (q = 1.5E-05) and COX7C (q = 0.0066) from Complex 4 and ATP5K (q = 0.041, Complex 5). Quetiapine increased the expression of COX6A2 (by 19 ± 1%, q = 5.59E-05, Complex 4), while lithium increased the expression of UQCRQ (by 20 ± 3%, q = 0.049, Complex 3) and COX6A2 (by 8 ± 2%, q = 0.00013, Complex 4).
Control release of mitochondria-targeted antioxidant by injectable self-assembling peptide hydrogel ameliorated persistent mitochondrial dysfunction and inflammation after acute kidney injury
Published in Drug Delivery, 2018
Meng Zhao, Yijie Zhou, Shuyun Liu, Lan Li, Younan Chen, Jingqiu Cheng, Yanrong Lu, Jingping Liu
Although the extended release of MT in KLDD hydrogel was confirmed in vitro, but the effect of slow release of MT on AKI has not been assayed before. IRI was a common cause of AKI with intricate pathological mechanisms such as oxidative stress, mitochondrial injury, and inflammatory response (Zuk & Bonventre, 2016). Oxidative stress upon IR caused direct injury to mitochondria and led to ATP depletion, inflammasome release, and apoptotic cell death after IRI (Loor et al., 2011; Bhargava & Schnellmann, 2017). Thus, we first evaluated the protective role of MT-loaded SAP hydrogel on renal mitochondria injury in IRI mice. PGC-1α was a master regulator of mitochondrial biogenesis and is known to associate with transcription factors responsible for mitochondrial genes such as TFAM expression. Our results showed that IRI mice had increased renal mtROS, mitochondrial fragments, and decline of key mitochondrial biogenesis-related genes such as ATP5a-1, PGC-1α, and TFAM at five days post AKI. These results were highly similar with previous report, which observed renal mitochondria damage even at 2.5 months in AKI rats (Szeto et al., 2017). However, free MT slightly reduced renal mitochondria injury and only improved a few parameters such as mitochondrial complex I subunits (NDUFS8) expression. In contrast, SAP-MT significantly reduced renal mtROS production, enhanced mitochondrial architecture, and almost biogenesis-related genes expression in IRI mice. The possible reason was that single injected free MT was rapidly eliminated in vivo and thus could not provide sustained protection as SAP-MT on renal mitochondria after IRI.
Visual Observation of Abdominal Adhesion Progression Based on an Optimized Mouse Model of Postoperative Abdominal Adhesions
Published in Journal of Investigative Surgery, 2023
Zijun Wang, Enmeng Li, Cancan Zhou, Bolun Qu, Tianli Shen, Jie Lian, Gan Li, Yiwei Ren, Yunhua Wu, Qinhong Xu, Guangbing Wei, Xuqi Li
On POD 3, direct interactions between Ndufs3, Ndufs6, Ndufs8, Ndufa8, Uqcrfs1 and other targets were predicted, and the enrichment analysis indicated that the top 10 hub genes were significantly enriched in electron transfer activity, the respirasome and the mitochondrial respirasome (Figure 8B). In vivo, reactive oxygen species (ROS) are mainly derived from mitochondrial respiratory chain function and are indicators of the oxidative stress state in vivo. These results suggested that the formation of PAAs was related to oxidative stress on POD 3.