Results 11 to 20 of about 289,389 (237)

Hepatic Form of Dihydrolipoamide Dehydrogenase Deficiency (DLDD): Phenotypic Spectrum, Laboratory Findings, and Therapeutic Approaches in 52 Patients. [PDF]

open access: hybridJ Inherit Metab Dis
Dihydrolipoamide dehydrogenase deficiency (MIM 246900/DLDD) is an autosomal recessive mitochondrial disease with three clinical subgroups. The hepatic form leads to recurrent metabolic decompensations often accompanied by elevated levels of liver ...
Hammann N   +18 more
europepmc   +4 more sources

Roles of Dihydrolipoamide Dehydrogenase in Health and Disease. [PDF]

open access: yesAntioxid Redox Signal, 2023
SIGNIFICANCE Dihydrolipoamide dehydrogenase (DLDH) is a flavin-dependent disulfide oxidoreductase. The active form of DLDH is a stable homodimer and its deficiencies have been linked to numerous metabolic disorders. A better understanding of DLDH's redox
Yan LJ, Wang Y.
europepmc   +4 more sources

The Effects of a Ketogenic Diet on Patients with Dihydrolipoamide Dehydrogenase Deficiency. [PDF]

open access: yesNutrients, 2021
Background: Dihydrolipoamide dehydrogenase (DLD lipoamide dehydrogenase, the E3 subunit of the pyruvate dehydrogenase complex (PDHC)) is the third catalytic enzyme of the PDHC, which converts pyruvate to acetyl-CoA catalyzed with the introduction of ...
Staretz-Chacham O   +7 more
europepmc   +5 more sources

Dihydrolipoamide dehydrogenase of Vibrio splendidus is involved in adhesion to Apostichopus japonicus [PDF]

open access: yesVirulence, 2019
Vibrio splendidus is one of the most opportunistic marine pathogens and infects many important marine animals, including the sea cucumber Apostichopus japonicus.
Fa Dai   +6 more
doaj   +3 more sources

In depth profiling of dihydrolipoamide dehydrogenase deficiency in primary patients fibroblasts reveals metabolic reprogramming secondary to mitochondrial dysfunction [PDF]

open access: yesMolecular Genetics and Metabolism Reports
Dihydrolipoamide dehydrogenase (DLD) deficiency is an autosomal recessive disorder characterized by a functional disruption in several critical mitochondrial enzyme complexes, including pyruvate dehydrogenase and α-ketoglutarate dehydrogenase.
Uri Sprecher   +8 more
doaj   +3 more sources

Chronic Inhibition of Mitochondrial Dihydrolipoamide Dehydrogenase (DLDH) as an Approach to Managing Diabetic Oxidative Stress [PDF]

open access: yesAntioxidants, 2019
Mitochondrial dihydrolipoamide dehydrogenase (DLDH) is a redox enzyme involved in decarboxylation of pyruvate to form acetyl-CoA during the cascade of glucose metabolism and mitochondrial adenine triphosphate (ATP) production.
Xiaojuan Yang, Jing Song, Liang-Jun Yan
doaj   +3 more sources

Newborn screening for dihydrolipoamide dehydrogenase deficiency: Citrulline as a useful analyte [PDF]

open access: yesMolecular Genetics and Metabolism Reports, 2014
Dihydrolipoamide dehydrogenase deficiency, also known as maple syrup urine disease (MSUD) type III, is caused by the deficiency of the E3 subunit of branched chain alpha-ketoacid dehydrogenase (BCKDH), α-ketoglutarate dehydrogenase (αKGDH), and pyruvate ...
Shane C. Quinonez   +4 more
doaj   +4 more sources

RGD-modified dihydrolipoamide dehydrogenase conjugated to titanium dioxide nanoparticles - switchable integrin-targeted photodynamic treatment of melanoma cells. [PDF]

open access: goldRSC Adv, 2018
The photocytotoxic effect of UVA-excited titanium dioxide (TiO2), which is caused by the generation of reactive oxygen species (ROS), is often used in medical applications, such as cancer treatment. Photodynamic-therapy (PDT) is applied in several cancer
Dayan A, Fleminger G, Ashur-Fabian O.
europepmc   +4 more sources

Structural and Biochemical Investigation of Selected Pathogenic Mutants of the Human Dihydrolipoamide Dehydrogenase. [PDF]

open access: yesInt J Mol Sci, 2023
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 ...
Szabo E   +8 more
europepmc   +2 more sources

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