Dihydrolipoamide dehydrogenase - Wikipedia
Dihydrolipoamide dehydrogenase (DLD), also known as dihydrolipoyl dehydrogenase, mitochondrial, is an enzyme that in humans is encoded by the DLD gene. [5] [6] [7] [8] DLD is a flavoprotein enzyme that oxidizes dihydrolipoamide to lipoamide.
Lipoamide dehydrogenase (LADH) deficiency: medical …
2023年2月26日 · (Dihydro)lipoamide dehydrogenase (LADH) deficiency is an autosomal recessive genetic metabolic disorder. It generally presents with an onset in the neonatal age and premature death. The clinical picture usually involves metabolic decompensation and lactic acidosis that lead to neurological, cardiological, and/or hepatological outcomes.
Lipoamide dehydrogenase - SpringerLink
In this review the structure and function of the lipoamide dehydrogenase component of the keto acid dehydrogenase complexes is discussed. Three structural models are available and in recent years many new genes have been sequenced and expressed. Unsolved is the role...
Dihydrolipoamide dehydrogenase, pyruvate oxidation, and …
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. The worldwide used antiepileptic drug valproic acid (VPA) may ...
二氢硫辛酰胺脱氢酶 - 百度百科
二氢硫辛酰胺脱氢酶（EC1.8.1.4，英语：Dihydrolipoamide dehydrogenase，缩写DLD，又称为线粒体二氢硫辛酸脱氢酶，dihydrolipoyl dehydrogenase, mitochondrial）是一种由人类基因DLD所编码的黄素蛋白，其作用是将二氢硫辛酰胺脱氢从而转化为氧化型硫辛酰胺。
Dihydrolipoamide Dehydrogenase Deficiency - GeneReviews® - NCBI Bookshelf
2014年7月17日 · Dihydrolipoamide dehydrogenase (DLD) functions as the E3 subunit in three mitochondrial enzyme complexes (BCKDH, αKGDH, PDH) and the L protein of the glycine cleavage system. As the E3 subunit, it catalyzes the oxidative regeneration of the lipoic acid covalently bound to the E2 subunit, generating NADH in the process.
Lipoamide - an overview | ScienceDirect Topics
Lipoamide dehydrogenase (NADH 2: lipoamide oxidoreductase, EC 1.6.4.3) is the flavoprotein first obtained from pig heart by Straub (1939) and called by him “diaphorase”.
DLD Gene - GeneCards | DLDH Protein | DLDH Antibody
2024年10月1日 · DLD (Dihydrolipoamide Dehydrogenase) is a Protein Coding gene. Diseases associated with DLD include Dihydrolipoamide Dehydrogenase Deficiency and Methemoglobinemia Due To Deficiency Of Methemoglobin Reductase. Among its related pathways are Leucine, isoleucine and valine metabolism and 2-oxobutanoate degradation.
Protein lipoylation: An evolutionarily conserved metabolic …
For example, the pyruvate dehydrogenase (PDH) and alpha-ketoglutarate (KDH) complexes regulate distinct carbon entry points into the central metabolic pathway of the TCA cycle. On both complexes, lipoylation is critical for proper enzyme function, and removal of this modification is part of a cellular mechanism to inhibit their activities.
Human dihydrolipoamide dehydrogenase (E3) deficiency: Novel …
2018年7月1日 · 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 moonlighting biochemical phenomena related to the affected (dihydro)lipoamide dehydrogenase (LADH, E3, gene: dld), in particular the generation of reactive oxygen species (ROS).