Cargando…

Structural and Biochemical Investigation of Selected Pathogenic Mutants of the Human Dihydrolipoamide Dehydrogenase

Clinically relevant disease-causing variants of the human dihydrolipoamide dehydrogenase (hLADH, hE3), a common component of the mitochondrial α-keto acid dehydrogenase complexes, were characterized using a multipronged approach to unravel the molecular pathomechanisms that underlie hLADH deficiency...

Descripción completa

Detalles Bibliográficos
Autores principales:	Szabo, Eszter, Nemes-Nikodem, Eva, Vass, Krisztina Rubina, Zambo, Zsofia, Zrupko, Eszter, Torocsik, Beata, Ozohanics, Oliver, Nagy, Balint, Ambrus, Attila
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	MDPI 2023
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10341545/ https://www.ncbi.nlm.nih.gov/pubmed/37446004 http://dx.doi.org/10.3390/ijms241310826

Ejemplares similares

Biochemical characterization of patients with dihydrolipoamide dehydrogenase deficiency
por: Wongkittichote, Parith, et al.
Publicado: (2023)

An Updated View on the Molecular Pathomechanisms of Human Dihydrolipoamide Dehydrogenase Deficiency in Light of Novel Crystallographic Evidence
por: Ambrus, Attila
Publicado: (2019)

Biochemical and structural characterization of the apicoplast dihydrolipoamide dehydrogenase of Plasmodium falciparum
por: Laine, Larissa M., et al.
Publicado: (2015)

The phenotypic spectrum of dihydrolipoamide dehydrogenase deficiency in Saudi Arabia
por: Alfarsi, Anar, et al.
Publicado: (2021)

Mitochondrial Dihydrolipoamide Dehydrogenase Is Upregulated in Response to Intermittent Hypoxic Preconditioning
por: Li, Rongrong, et al.
Publicado: (2015)

Dihydrolipoamide dehydrogenase of Vibrio splendidus is involved in adhesion to Apostichopus japonicus
por: Dai, Fa, et al.
Publicado: (2019)

The Effects of a Ketogenic Diet on Patients with Dihydrolipoamide Dehydrogenase Deficiency
por: Staretz-Chacham, Orna, et al.
Publicado: (2021)

Current Approaches in Molecular Enzymology
por: Szabo, Eszter, et al.
Publicado: (2022)

Newborn screening for dihydrolipoamide dehydrogenase deficiency: Citrulline as a useful analyte
por: Quinonez, Shane C., et al.
Publicado: (2014)

Attenuation of radiation toxicity by the phosphine resistance factor dihydrolipoamide dehydrogenase (DLD)
por: Alzahrani, Saad M., et al.
Publicado: (2019)

Identification of Dihydrolipoamide Dehydrogenase as Potential Target of Vemurafenib-Resistant Melanoma Cells
por: Tabolacci, Claudio, et al.
Publicado: (2022)

Probing the E1o-E2o and E1a-E2o Interactions in Binary Subcomplexes of the Human 2-Oxoglutarate Dehydrogenase and 2-Oxoadipate Dehydrogenase Complexes by Chemical Cross-Linking Mass Spectrometry and Molecular Dynamics Simulation
por: Ozohanics, Oliver, et al.
Publicado: (2023)

Dihydrolipoamide dehydrogenase regulates cystine deprivation-induced ferroptosis in head and neck cancer
por: Shin, Daiha, et al.
Publicado: (2020)

Structural alterations by five disease-causing mutations in the low-pH conformation of human dihydrolipoamide dehydrogenase (hLADH) analyzed by molecular dynamics – Implications in functional loss and modulation of reactive oxygen species generation by pathogenic hLADH forms
por: Ambrus, Attila, et al.
Publicado: (2015)

Chronic Inhibition of Mitochondrial Dihydrolipoamide Dehydrogenase (DLDH) as an Approach to Managing Diabetic Oxidative Stress
por: Yang, Xiaojuan, et al.
Publicado: (2019)

Hydrogen-Deuterium Exchange Mass Spectrometry: A Novel Structural Biology Approach to Structure, Dynamics and Interactions of Proteins and Their Complexes
por: Ozohanics, Oliver, et al.
Publicado: (2020)

Inhibiting Sperm Pyruvate Dehydrogenase Complex and Its E3 Subunit, Dihydrolipoamide Dehydrogenase Affects Fertilization in Syrian Hamsters
por: Siva, Archana B., et al.
Publicado: (2014)

Rearrangement of mitochondrial pyruvate dehydrogenase subunit dihydrolipoamide dehydrogenase protein–protein interactions by the MDM2 ligand nutlin‐3
por: Way, Luke, et al.
Publicado: (2016)

Catalysis of transthiolacylation in the active centers of dihydrolipoamide acyltransacetylase components of 2‐oxo acid dehydrogenase complexes
por: Chakraborty, Joydeep, et al.
Publicado: (2018)

Redox status of cysteines does not alter functional properties of human dUTPase but the Y54C mutation involved in monogenic diabetes decreases protein stability
por: Szabó, Judit Eszter, et al.
Publicado: (2021)

Author Correction: Redox status of cysteines does not alter functional properties of human dUTPase but the Y54C mutation involved in monogenic diabetes decreases protein stability
por: Szabó, Judit Eszter, et al.
Publicado: (2021)

Dichloroacetate and thiamine improve survival and mitochondrial stress in a C. elegans model of dihydrolipoamide dehydrogenase deficiency
por: Broxton, Chynna N., et al.
Publicado: (2022)

Pharmacological Blockade of Cannabinoid CB(1) Receptors in Diet-Induced Obesity Regulates Mitochondrial Dihydrolipoamide Dehydrogenase in Muscle
por: Arrabal, Sergio, et al.
Publicado: (2015)

Possible Biochemical Processes Underlying the Positive Health Effects of Plant-Based Diets—A Narrative Review
por: Szabo, Zoltan, et al.
Publicado: (2021)

RGD-modified dihydrolipoamide dehydrogenase conjugated to titanium dioxide nanoparticles – switchable integrin-targeted photodynamic treatment of melanoma cells
por: Dayan, Avraham, et al.
Publicado: (2018)

Bio-Fenton reaction involved in the cleavage of the ethoxylate chain of nonionic surfactants by dihydrolipoamide dehydrogenase from Pseudomonas nitroreducens TX1
por: Hung, Kuo-Chan, et al.
Publicado: (2019)

Targeted tumor therapy by Rubia tinctorum L.: analytical characterization of hydroxyanthraquinones and investigation of their selective cytotoxic, adhesion and migration modulator effects on melanoma cell lines (A2058 and HT168-M1)
por: Lajkó, Eszter, et al.
Publicado: (2015)

Emerging azithromycin-resistance among the Neisseria gonorrhoeae strains isolated in Hungary
por: Brunner, Alexandra, et al.
Publicado: (2016)

Fabrication of Submicrometer-Sized Meloxicam Particles Using Femtosecond Laser Ablation in Gas and Liquid Environments
por: Nagy, Eszter, et al.
Publicado: (2021)

Functional Versatility of the Human 2-Oxoadipate Dehydrogenase in the L-Lysine Degradation Pathway toward Its Non-Cognate Substrate 2-Oxopimelic Acid
por: Nemeria, Natalia S., et al.
Publicado: (2022)

Validation and translation of the Hungarian version of the Australian Pelvic Floor Questionnaire (APFQ-H)
por: Hock, Márta, et al.
Publicado: (2022)

Recombination Events Shape the Genomic Evolution of Infectious Bronchitis Virus in Europe
por: Bali, Krisztina, et al.
Publicado: (2021)

Pseudomonas aeruginosa Uses Dihydrolipoamide Dehydrogenase (Lpd) to Bind to the Human Terminal Pathway Regulators Vitronectin and Clusterin to Inhibit Terminal Pathway Complement Attack
por: Hallström, Teresia, et al.
Publicado: (2015)

Suppression of a core metabolic enzyme dihydrolipoamide dehydrogenase (dld) protects against amyloid beta toxicity in C. elegans model of Alzheimer's disease
por: Ahmad, Waqar, et al.
Publicado: (2020)

Rg3 regulates myocardial pyruvate metabolism via P300-mediated dihydrolipoamide dehydrogenase 2-hydroxyisobutyrylation in TAC-induced cardiac hypertrophy
por: Ni, Jingyu, et al.
Publicado: (2022)

Corrigendum: Genetic diversity of imported PRRSV-2 strains, 2005–2020, Hungary
por: Jakab, Szilvia, et al.
Publicado: (2022)

Genetic diversity of imported PRRSV-2 strains, 2005–2020, Hungary
por: Jakab, Szilvia, et al.
Publicado: (2022)

Discovery of Novel Dihydrolipoamide S-Succinyltransferase Inhibitors Based on Fragment Virtual Screening
por: Wei, Chengqian, et al.
Publicado: (2021)

Proteomic Changes of Osteoclast Differentiation in Rheumatoid and Psoriatic Arthritis Reveal Functional Differences
por: Kovács, Orsolya Tünde, et al.
Publicado: (2022)

The Role of Number Notation: Sign-Value Notation Number Processing is Easier than Place-Value
por: Krajcsi, Attila, et al.
Publicado: (2012)

Cannot write session to /tmp/vufind_sessions/sess_f2q4m9ioibdg1jpeba4prvmftk