Results 101 to 110 of about 60,467 (293)

Clinical syndromes associated with Coenzyme Q10 deficiency. [PDF]

, 2018
Primary Coenzyme Q deficiencies represent a group of rare conditions caused by mutations in one of the genes required in its biosynthetic pathway at the enzymatic or regulatory level.
Alc\ue1zar-Fabra, Maria, Brea-Calvo, Gloria, Trevisson, Eva   +2 more
core   +1 more source

Targeting the CMKLR1‐Mediated Signaling Rebalances Immunometabolism State in Middle‐Age Testicular Macrophages

Advanced Science, EarlyView.
In middle age, testicular CMKLR1⁺ macrophages exhibited a pro‐inflammatory immunometabolic profile, mediated by adipose signals associated with high BMI. However, inhibition of CMKLR1 signaling, either through Cmklr1 genetic ablation or treatment with a CMKLR1 antagonist peptide, can reverse this phenotype.
Zhendong Zhu, Feifei Du, Yang Liu, Jinchunzi Yang, Lei Ge, Tianxia Xiao, Mengxia Li, Jie Chen, Jia Tang, Yali Yang, Jian V. Zhang   +10 more
wiley   +1 more source

Disruption of mitochondrial energy metabolism is a putative pathogenesis of Diamond-Blackfan anemia

iScience
Summary: Energy metabolism in the context of erythropoiesis and related diseases remains largely unexplored. Here, we developed a primary cell model by differentiating hematopoietic stem progenitor cells toward the erythroid lineage and suppressing the ...
Rudan Xiao, Lijuan Zhang, Zijuan Xin, Junwei Zhu, Qian Zhang, Guangmin Zheng, Siyun Chu, Jing Wu, Lu Zhang, Yang Wan, Xiaojuan Chen, Weiping Yuan, Zhaojun Zhang, Xiaofan Zhu, Xiangdong Fang   +14 more
doaj   +1 more source

Targeting Lactate and Lactylation in Cancer Metabolism and Immunotherapy

Advanced Science, EarlyView.
Lactate, once deemed a metabolic waste, emerges as a central regulator of cancer progression. This review elucidates how lactate and its epigenetic derivative, protein lactylation, orchestrate tumor metabolism, immune suppression, and therapeutic resistance.
Jiajing Gong, Yuhang Xie, Zhijie Weng, Yongzhi Wu, Longjiang Li, Bo Li   +5 more
wiley   +1 more source

Signal transducer and activator of transcription 2 deficiency is a novel disorder of mitochondrial fission [PDF]

, 2015
Defects of mitochondrial dynamics are emerging causes of neurological disease. In two children presenting with severe neurological deterioration following viral infection we identified a novel homozygous STAT2 mutation, c.1836C4A (p.Cys612Ter), using ...
Anderson, G, Cale, CM, Chalasani, A, Chan, E, Duchen, MR, Gilmour, KC, Hambleton, S, Jacques, S, Osellame, LD, Plagnol, V, Qasim, W, Rahman, S, Shahni, R, Taanman, JW, Wadatilake, Y   +14 more
core   +1 more source

Connexin43 Deficiency Leads to Ventricular Arrhythmias by Reprogramming Proline Metabolism

Advanced Science, EarlyView.
The study demonstrated that connexin43 (Cx43) knockout caused arrhythmic phenotype and decreased proline content in vitro and in vivo. Mechanistically, Cx43 interacts with the amino acid transporter SNAT2 (sodium‐dependent neutral amino acid transporter), and its deficiency disrupts proline transport and metabolism.
Hangying Ying, Hangping Fan, Yunhe Wang, Ruhong Jiang, Dongsheng Cai, Hui Cheng, Hao Wang, Chenyang Jiang, Ping Liang   +8 more
wiley   +1 more source

A genetic toolkit for the analysis of metabolic changes in Drosophila provides new insights into metabolic responses to stress and malignant transformation [PDF]

, 2019
Regulation of the energetic metabolism occurs fundamentally at the cellular level, so analytical strategies must aim to attain single cell resolution to fully embrace its inherent complexity.
Behrensen, C., Durrieu, Lucía, Gándara, Lautaro, Wappner, Pablo   +3 more
core   +1 more source

Oxphos Targeting of Mycn -Amplified Neuroblastoma

Frontiers in Oncology
Abstract Summary High risk - neuroblastoma (HR-NB) is a pediatric solid tumor with high lethality. Half of HR-NB are driven byMYCNgene amplification (MNA). These HR-NBs require high dosage chemotherapy and often relapse. Moreover, current therapies can cause severe long-term side effects and new therapies are urgently needed. This study investigates a
Soraya Epp, Donagh Egan, Evon Poon, Amirah Adlina Abdul Aziz, Kieran Wynne, Louis Chesler, Melinda Halasz, Walter Kolch   +7 more
openaire   +1 more source

3D Bioprinted Fat‐Myocardium Model Unravels the Role of Adipocyte Hypertrophy in Atrial Dysfunction

Advanced Science, EarlyView.
A human‐derived 3D bioprinted fat–myocardium model is developed to investigate how adipocyte hypertrophy drives atrial dysfunction in obesity. Palmitate‐induced adipocyte hypertrophy promotes adipose dysfunction that impairs atrial cardiomyocyte metabolism and electrophysiology through both paracrine and direct interactions. This platform recapitulates
Lara Ece Celebi, Pinar Zorlutuna
wiley   +1 more source

Metabolic signaling directs the reciprocal lineage decisions of αβ and γδ T cells [PDF]

, 2018
Wiring metabolic signaling circuits in thymocytes Cell differentiation is often accompanied by metabolic changes. Yang et al. report that generation of double-positive (DP) thymocytes from double-negative (DN) cells coincides with dynamic regulation of ...
Awad, Walid, Blanco, Daniel Bastardo, Chen, Wenan, Chen, Xiang, Cheng, Changde, Chi, Hongbo, Dash, Pradyot, Dhungana, Yogesh, Easton, John, Guo, Xi-Zhi J., Kc, Anil, Neale, Geoffrey, Rosencrance, Celeste, Thomas, Paul G., Yang, Kai   +14 more
core   +1 more source