Results 41 to 50 of about 22,770 (160)

Evaluating the association of biallelic OGDHL variants with significant phenotypic heterogeneity

Genome Medicine, 2023
Background Biallelic variants in OGDHL, encoding part of the α-ketoglutarate dehydrogenase complex, have been associated with highly heterogeneous neurological and neurodevelopmental disorders.
Sheng-Jia Lin, Barbara Vona, Tracy Lau, Kevin Huang, Maha S. Zaki, Huda Shujaa Aldeen, Ehsan Ghayoor Karimiani, Clarissa Rocca, Mahmoud M. Noureldeen, Ahmed K. Saad, Cassidy Petree, Tobias Bartolomaeus, Rami Abou Jamra, Giovanni Zifarelli, Aditi Gotkhindikar, Ingrid M. Wentzensen, Mingjuan Liao, Emalyn Elise Cork, Pratishtha Varshney, Narges Hashemi, Mohammad Hasan Mohammadi, Aboulfazl Rad, Juanita Neira, Mehran Beiraghi Toosi, Cordula Knopp, Ingo Kurth, Thomas D. Challman, Rebecca Smith, Asmahan Abdalla, Thomas Haaf, Mohnish Suri, Manali Joshi, Wendy K. Chung, Andres Moreno-De-Luca, Henry Houlden, Reza Maroofian, Gaurav K. Varshney   +36 more
doaj   +1 more source

Dysregulation of mitochondrial α-ketoglutarate dehydrogenase leads to elevated lipid peroxidation in CHCHD2-linked Parkinson’s disease models

Nature Communications
Dysregulation of mitochondrial function has been implicated in Parkinson’s disease (PD), but the role of mitochondrial metabolism in disease pathogenesis remains to be elucidated.
Ge Gao, Yong Shi, Han-Xiang Deng, Dimitri Krainc   +3 more
doaj   +1 more source

AZACYTIDINE (A) ASSOCIATED WITH VITAMINS (B1, C, AND D) IN THE TREATMENT OF ACUTE MYELOID LEUKEMIA.

Hematology, Transfusion and Cell Therapy, 2023
Objective: To report the clinical evolution of AML with complex karyotype in an elderly individual using the AZAVIT-ABCDEF protocol. Method: Information was obtained through electronic medical record review (HSPE–IAMSPE-SP).
R Conserva, VM Sthel, VLP Figueiredo
doaj   +1 more source

ACUTE MYELOID LEUKEMIA IN COMPLETE RESPONSE FOR 2 YEARS WITH AZACYTIDINE ASSOCIATED WITH HIGH-DOSE VITAMINS

Hematology, Transfusion and Cell Therapy, 2023
Acute Myeloid Leukemia (AML) is an uncommon neoplasia, and its treatment is challenging. The AZAVIT-ABCEF protocol (Plataforma Brasil-CAAE: 53015421.0.0000.5463) is of low intensity and reasonably well-tolerated. Objective: To report a clinical case of a
VM Sthel, VLP Figueiredo
doaj   +1 more source

Pseudomonas aeruginosa MifS-MifR Two-Component System Is Specific for α-Ketoglutarate Utilization. [PDF]

PLoS ONE, 2015
Pseudomonas aeruginosa is a Gram-negative, metabolically versatile opportunistic pathogen that elaborates a multitude of virulence factors, and is extraordinarily resistant to a gamut of clinically significant antibiotics.
Gorakh Tatke, Hansi Kumari, Eugenia Silva-Herzog, Lourdes Ramirez, Kalai Mathee   +4 more
doaj   +1 more source

Isocitrate dehydrogenase mutation in Vibrio anguillarum results in virulence attenuation and immunoprotection in rainbow trout (Oncorhynchus mykiss)

BMC Microbiology, 2017
Background Vibrio anguillarum is an extracellular bacterial pathogen that is a causative agent of vibriosis in finfish and crustaceans with mortality rates ranging from 30% to 100%.
Xiangyu Mou, Edward J. Spinard, Shelby L. Hillman, David R. Nelson   +3 more
doaj   +1 more source

Abnormal Pyruvate and α-Ketoglutarate Dehydrogenase Complexes in a Patient with Lactic Acidemia [PDF]

Pediatric Research, 1979
Activities of pyruvate decarboxylase (PDC), alpha-ketoglutarate decarboxylase (KGDC) and both the pyruvate and alpha-ketoglutarate dehydrogenase complexes (PDH complex and KGDH complex) were measured, and kinetic properties of PDC were studied in fibroblasts derived from normal individuals and from a 2-yr-old girl with congenital lactic acidemia and ...
Y, Kuroda, J J, Kline, L, Sweetman, W L, Nyhan, T D, Groshong   +4 more
openaire   +2 more sources

The emerging importance of the α-keto acid dehydrogenase complexes in serving as intracellular and intercellular signaling platforms for the regulation of metabolism

Redox Biology
The α-keto acid dehydrogenase complex (KDHc) class of mitochondrial enzymes is composed of four members: pyruvate dehydrogenase (PDHc), α-ketoglutarate dehydrogenase (KGDHc), branched-chain keto acid dehydrogenase (BCKDHc), and 2-oxoadipate dehydrogenase
Ryan J. Mailloux
doaj   +1 more source

Interaction between NAD-dependent isocitrate dehydrogenase, alpha-ketoglutarate dehydrogenase complex, and NADH:ubiquinone oxidoreductase.

Journal of Biological Chemistry, 1987
Interaction between the alpha-ketoglutarate dehydrogenase complex and NAD+-dependent isocitrate dehydrogenase was detected with a variety of techniques including polyethylene glycol precipitation, ultracentrifugation, and centrifugal gel filtration on a Sepharose 6B column.
Z, Porpaczy, B, Sumegi, I, Alkonyi
openaire   +2 more sources

GLS2 inhibition synergizes with copper to reprogram TCA cycle for cuproptosis-driven radiosensitization in esophageal cancer

Experimental Hematology & Oncology
Esophageal squamous cell carcinoma (ESCC) is notorious for its poor prognosis. In the present study, the role of glutaminase 2 (GLS2) and copper (Cu) in the radiosensitivity of ESCC was explored.
Wang Jing, Wenhao Wang, Yi Ding, Renya Zeng, Hui Zhu, Zhichao Kang, Alei Feng, Zhe Yang   +7 more
doaj   +1 more source