Human dihydrolipoamide dehydrogenase (E3) deficiency: Novel insights into the structural basis and molecular pathomechanism

A Ambrus, V Adam-Vizi - Neurochemistry international, 2018 - Elsevier
This review summarizes our present view on the molecular pathogenesis of human (h) E3-
deficiency caused by a variety of genetic alterations with a special emphasis on the …

Influence of disease-causing mutations on protein structural networks

VM Prabantu, N Naveenkumar… - Frontiers in molecular …, 2021 - frontiersin.org
The interactions between residues in a protein tertiary structure can be studied effectively
using the approach of protein structure network (PSN). A PSN is a node-edge representation …

Downregulation of dihydrolipoyl dehydrogenase by UVA suppresses melanoma progression via triggering oxidative stress and altering energy metabolism

S Yumnam, MC Kang, SH Oh, HC Kwon, JC Kim… - Free Radical Biology …, 2021 - Elsevier
Melanoma, the most severe form of skin cancer, has poor prognosis and is resistant to
chemotherapy. Targeting cancer metabolism is a promising approach in cancer …

Comprehensive analysis of the cuproptosis-related gene DLD across cancers: A potential prognostic and immunotherapeutic target

W Yang, Q Guo, H Wu, L Tong, J **ao… - Frontiers in …, 2023 - frontiersin.org
DLD is a key gene involved in “cuproptosis,” but its roles in tumor progression and immunity
remain unclear. Exploring the potential mechanisms and biological roles of DLD may …

[HTML][HTML] Structure of the dihydrolipoamide succinyltransferase (E2) component of the human alpha-ketoglutarate dehydrogenase complex (hKGDHc) revealed by cryo …

B Nagy, M Polak, O Ozohanics, Z Zambo… - … et Biophysica Acta (BBA …, 2021 - Elsevier
Background The human mitochondrial alpha-ketoglutarate dehydrogenase complex
(hKGDHc) converts KG to succinyl-CoA and NADH. Malfunction of and reactive oxygen …

Dihydrolipoamide dehydrogenase, pyruvate oxidation, and acetylation-dependent mechanisms intersecting drug iatrogenesis

IF Duarte, J Caio, MF Moedas, LA Rodrigues… - Cellular and Molecular …, 2021 - Springer
In human metabolism, pyruvate dehydrogenase complex (PDC) is one of the most intricate
and large multimeric protein systems representing a central hub for cellular homeostasis …

Underlying molecular alterations in human dihydrolipoamide dehydrogenase deficiency revealed by structural analyses of disease-causing enzyme variants

E Szabo, P Wilk, B Nagy, Z Zambo, D Bui… - Human molecular …, 2019 - academic.oup.com
Human dihydrolipoamide dehydrogenase (hLADH, hE3) deficiency (OMIM# 246900) is an
often prematurely lethal genetic disease usually caused by inactive or partially inactive hE3 …

[HTML][HTML] A multipronged approach unravels unprecedented protein–protein interactions in the human 2-oxoglutarate dehydrogenase multienzyme complex

J Zhou, L Yang, O Ozohanics, X Zhang, J Wang… - Journal of Biological …, 2018 - Elsevier
The human 2-oxoglutaric acid dehydrogenase complex (hOGDHc) plays a pivotal role in the
tricarboxylic acid (TCA) cycle, and its diminished activity is associated with …