Ask about this productRelated genes to: UQCRC1 antibody
- Gene:
- UQCRC1 NIH gene
- Name:
- ubiquinol-cytochrome c reductase core protein 1
- Previous symbol:
- -
- Synonyms:
- D3S3191, QCR1, UQCR1
- Chromosome:
- 3p21.31
- Locus Type:
- gene with protein product
- Date approved:
- 1993-07-09
- Date modifiied:
- 2017-09-12
Related products to: UQCRC1 antibody
Related articles to: UQCRC1 antibody
- The anterior cingulate cortex (ACC), crucial for executive function, is frequently impaired in progressive supranuclear palsy (PSP), yet mechanisms underlying this selective vulnerability remain unclear. Given the integration of astrocytes into neural circuits, we hypothesized that astrocyte dysfunction and altered astrocyte-neuron crosstalk contribute to functional abnormalities in the ACC in PSP. To test this hypothesis, we conducted a multimodal analysis integrating SWATH-MS-based proteomics, histopathology, and in vivo magnetic resonance spectroscopy (MRS) in postmortem and living brains of patients with PSP and healthy controls (HCs). The astrocytic markers glial fibrillary acidic protein (GFAP) and aquaporin-4 (AQP4) were significantly elevated in the ACC of patients with PSP compared with those in HCs. Enhanced astrocytic Ca⁺ signaling through the IP3-Ca⁺ cascade was suggested in the ACC of patients with PSP, consistent with elevated myo-inositol levels on MRS. Proteomic data revealed reduced expression of pyruvate dehydrogenase complex components (DLD and PDHX) and oxidative phosphorylation-related proteins, including astrocyte-enriched genes such as ETFDH and UQCRC1. MRS also revealed significantly increased levels of lactate and glutamate in the ACC of patients with PSP compared with those in HCs. Notably, myo-inositol, lactate, and glutamate levels were positively correlated, indicating astrocyte-associated metabolic dysfunction. Expression of glutamate-glutamine cycle-related molecules and neuronal markers was negatively correlated with GFAP and AQP4 levels, suggesting that astrocytic dysfunction is associated with alterations in the excitatory/inhibitory balance in the ACC of patients with PSP. These findings demonstrate that multiple aspects of astrocyte-neuron crosstalk, including AQP4-mediated glymphatic clearance, energy metabolism, and neurotransmitter cycling, are altered in the ACC of patients with PSP. Such disruptions may contribute to neuronal dysfunction. Our study highlights astrocyte dysfunction as a central feature of the PSP pathophysiology. - Source: PubMed
Publication date: 2026/04/24
Ono MaikoKumagai YutaHirata KoseiMaeda YukiAbe YurikaEndo HironobuShimizu HiroshiKakita AkiyoshiUchida YasuoTakado Yuhei - Fatty liver hemorrhagic syndrome (FLHS) is a nutrition-related metabolic disorder in laying hens characterized by excessive hepatic lipid accumulation and hemorrhagic lesions, leading to reduced productivity and increased mortality. However, the regulatory mechanisms linking mitochondrial dysfunction to hepatic lipid metabolism remain unclear. This study investigated the role of SIRT3 in modulating mitochondrial fatty acid oxidation during FLHS progression. An in vivo FLHS model was established by feeding laying hens with a high-energy, low-protein (HELP) diet, and an in vitro hepatic steatosis model was induced by free fatty acid (FFA) treatment in primary hepatocytes. Both models exhibited pronounced lipid accumulation in hepatic cells and altered hepatocellular injury-related parameters, which were associated with mitochondrial dysfunction and impaired fatty acid oxidation. Mechanistically, hepatic tissues and hepatocytes showed suppression of the SIRT3-AMPKα-PGC-1α signaling cascade, accompanied by reduced expression of mitochondrial biogenesis markers (NRF1, TFAM), impaired respiratory chain components (NDUFA9, SDHA, UQCRC1, COX4I1, ATP5B), and decreased transcription of fatty acid oxidation-related genes (PPARα, ACOX1, CPT1A, CPT2, ACADL, ACADM). Pharmacological activation of SIRT3 with AR-C17 restored AMPKα-PGC-1α signaling, enhanced mitochondrial biogenesis and respiratory function, and promoted fatty acid oxidation, thereby alleviating lipid accumulation in hepatocytes in both models. Collectively, these results demonstrate that SIRT3 is a key metabolic regulator maintaining mitochondrial oxidative function and lipid homeostasis in laying hens. Targeted activation of SIRT3 may provide a novel nutritional strategy for preventing or ameliorating FLHS and related metabolic disturbances in poultry production. - Source: PubMed
Publication date: 2026/04/07
Cao PanpanChen JinyanZeng ChunHu YangYuan JianyunGuo XiaoquanCao HuabinZhang CaiyingZhuang YuHu Guoliang - Ferroptosis plays a critical role in Parkinson's disease (PD). This study investigates the neuroprotective effect of a combination of two natural anthraquinones, rhein and emodin, against ferroptosis, focusing on the mitochondrial complex III subunit UQCRC1. Using network pharmacology and experimental validation in erastin/MPP-induced PC12 cells, we found that the optimal rhein-emodin combination potently inhibited ferroptotic cell death. This effect was associated with restored mitochondrial function, reduced lipid peroxidation, and the coordinated regulation of ferroptosis-related proteins, including the upregulation of GPX4 and FTH and downregulation of ACSL4. Crucially, the co-treatment markedly activated UQCRC1 expression. Furthermore, UQCRC1 knockdown abolished these anti-ferroptotic effects, establishing it as an essential mediator. Our findings demonstrate that the rhein-emodin combination attenuates ferroptosis primarily through UQCRC1 activation, highlighting its therapeutic promise and identifying UQCRC1 as a novel regulatory node for PD intervention. - Source: PubMed
Yusun AyiguzhaliWan HongmeiLi ShuangLiu YanpingChen HuaxianSun MoDing XudongZhang Chenning - Primary mitochondrial defects underlie the heterogeneity of many rare inherited disorders. Pathogenic variants that disrupt the function of the multi-subunit protein complexes of the mitochondrial respiratory chain contribute to a range of neurological phenotypes and other clinical manifestations. These variants are also thought to contribute to the onset and progression of numerous more common neurodegenerative conditions such as Parkinson's and Alzheimer's disease. Here we describe an individual affected with progressive muscle weakness and pain harboring a paternally inherited missense variant in , encoding a subunit of Complex III. Biochemical characterization of cells from the proband and his father demonstrated normal steady-state levels of UQCRC1 and UQCRC2 protein. Functional assessment of mitochondrial respiration in lymphoblasts and fibroblasts, however, showed a clear deficit in respiratory parameters in the proband, with a more attenuated response in the father. Lastly, we demonstrate that healthy mitochondria isolated from HEK293 cells can be transferred to the patient lymphoblasts, restoring basal mitochondrial respiration and ATP production. Perspectives on the contribution of this variant to the patient phenotypes, and the potential of mitochondrial transplantation and different compounds as treatment modalities for patients with primary mitochondrial deficits, is discussed. - Source: PubMed
Publication date: 2026/02/25
Piroli Gerardo GMyers RebeccaHolloway LyndaHayek AndrewLinebaugh EllenJones Julie RSkinner CindySkinner Steven AFrizzell NormaSteet Richard - Ischemic stroke (IS) and aging share similar pathophysiological features, including vascular dysfunction, inflammatory responses, and oxidative stress. However, the underlying molecular mechanisms remain unclear. This study aimed to identify key shared drive genes between IS and aging through integrated multi-omics analysis and machine learning approaches, and to explore their diagnostic and therapeutic potential. - Source: PubMed
Publication date: 2026/02/17
Wang PeiluWang YanWang DongliangLi YingxuanYin ZhenyuZhang Fuqing