Dihydrolipoamide dehydrogenase

DLD
Available structures
PDB	Ortholog search: PDBe RCSB
List of PDB id codes
	3RNM, 1ZMC, 1ZMD, 1ZY8, 2F5Z
Identifiers
Aliases	DLD, DLDD, DLDH, E3, GCSL, LAD, PHE3, dihydrolipoamide dehydrogenase, Dihydrolipoamide dehydrogenase, OGDC-E3
External IDs	OMIM: 238331; MGI: 107450; HomoloGene: 84; GeneCards: DLD; OMA:DLD - orthologs
Gene location (Human)
Chr.	Chromosome 7 (human)
End	107,931,730 bp
Gene location (Mouse)
Chr.	Chromosome 12 (mouse)
End	31,401,452 bp
RNA expression pattern
	Top expressed in
	right ventricle; ; biceps brachii; ; Skeletal muscle tissue of biceps brachii; ; thoracic diaphragm; ; Skeletal muscle tissue of rectus abdominis; ; vastus lateralis muscle; ; body of tongue; ; mucosa of sigmoid colon; ; jejunal mucosa; ; rectum;
	Top expressed in
	saccule; ; cardiac muscle tissue of left ventricle; ; otic placode; ; tail of embryo; ; extraocular muscle; ; intercostal muscle; ; soleus muscle; ; extensor digitorum longus muscle; ; interventricular septum; ; plantaris muscle;
	More reference expression data
	n/a
Gene ontology
Molecular function	flavin adenine dinucleotide binding; NAD binding; dihydrolipoyl dehydrogenase activity; oxidoreductase activity; pyruvate dehydrogenase (NAD+) activity; lipoamide binding; oxidoreductase activity, acting on a sulfur group of donors, NAD(P) as acceptor; electron transfer activity; protein binding;
Cellular component	oxoglutarate dehydrogenase complex; myelin sheath; pyruvate dehydrogenase complex; cilium; acrosomal matrix; nucleoplasm; mitochondrial matrix; mitochondrion; acrosomal vesicle; cytoplasmic vesicle; motile cilium; cell projection; nucleus;
Biological process	mitochondrial acetyl-CoA biosynthetic process from pyruvate; regulation of membrane potential; ageing; branched-chain amino acid catabolic process; tricarboxylic acid cycle; 2-oxoglutarate metabolic process; lysine catabolic process; regulation of acetyl-CoA biosynthetic process from pyruvate; cell redox homeostasis; proteolysis; lipoate metabolic process; dihydrolipoamide metabolic process; gastrulation; pyruvate metabolic process; sperm capacitation; acetyl-CoA biosynthetic process from pyruvate; mitochondrial electron transport, NADH to ubiquinone; cellular nitrogen compound metabolic process; histone succinylation;
	Sources:Amigo / QuickGO
Orthologs
	1738
	13382
	ENSG00000091140
	ENSMUSG00000020664
	P09622
	O08749
	NM_001289752; NM_000108; NM_001289750; NM_001289751
	NM_007861
	NP_000099; NP_001276679; NP_001276680; NP_001276681
	NP_031887
	Wikidata
View/Edit Human	View/Edit Mouse

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.

Dihydrolipoamide dehydrogenase (DLD) is a mitochondrial enzyme that plays a vital role in energy metabolism in eukaryotes. This enzyme is required for the complete reaction of at least five different multi-enzyme complexes.^[9] Additionally, DLD is a flavoenzyme oxidoreductase that contains a reactive disulfide bridge and a FAD cofactor that are directly involved in catalysis. The enzyme associates into tightly bound homodimers required for its enzymatic activity.^[10]

^ ^a ^b ^c GRCh38: Ensembl release 89: ENSG00000091140 – Ensembl, May 2017
^ ^a ^b ^c GRCm38: Ensembl release 89: ENSMUSG00000020664 – Ensembl, May 2017
^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^ "Entrez Gene: dihydrolipoamide dehydrogenase".
^ Otulakowski G, Robinson BH (December 1987). "Isolation and sequence determination of cDNA clones for porcine and human lipoamide dehydrogenase. Homology to other disulfide oxidoreductases". The Journal of Biological Chemistry. 262 (36): 17313–8. doi:10.1016/S0021-9258(18)45379-3. PMID 3693355.
^ Pons G, Raefsky-Estrin C, Carothers DJ, Pepin RA, Javed AA, Jesse BW, et al. (March 1988). "Cloning and cDNA sequence of the dihydrolipoamide dehydrogenase component human alpha-ketoacid dehydrogenase complexes". Proceedings of the National Academy of Sciences of the United States of America. 85 (5): 1422–6. Bibcode:1988PNAS...85.1422P. doi:10.1073/pnas.85.5.1422. PMC 279783. PMID 3278312.
^ Scherer SW, Otulakowski G, Robinson BH, Tsui LC (1991). "Localization of the human dihydrolipoamide dehydrogenase gene (DLD) to 7q31----q32". Cytogenetics and Cell Genetics. 56 (3–4): 176–7. doi:10.1159/000133081. hdl:10722/42531. PMID 2055113.
^ Babady NE, Pang YP, Elpeleg O, Isaya G (April 2007). "Cryptic proteolytic activity of dihydrolipoamide dehydrogenase". Proceedings of the National Academy of Sciences of the United States of America. 104 (15): 6158–63. Bibcode:2007PNAS..104.6158B. doi:10.1073/pnas.0610618104. PMC 1851069. PMID 17404228.
^ Ciszak EM, Makal A, Hong YS, Vettaikkorumakankauv AK, Korotchkina LG, Patel MS (January 2006). "How dihydrolipoamide dehydrogenase-binding protein binds dihydrolipoamide dehydrogenase in the human pyruvate dehydrogenase complex". The Journal of Biological Chemistry. 281 (1): 648–55. doi:10.1074/jbc.M507850200. PMID 16263718.

[refGRCh38Ensembl-1] GRCh38: Ensembl release 89: ENSG00000091140 – Ensembl, May 2017

[refGRCm38Ensembl-2] GRCm38: Ensembl release 89: ENSMUSG00000020664 – Ensembl, May 2017

[3] "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[4] "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[entrez-5] "Entrez Gene: dihydrolipoamide dehydrogenase".

[pmid3693355-6] Otulakowski G, Robinson BH (December 1987). "Isolation and sequence determination of cDNA clones for porcine and human lipoamide dehydrogenase. Homology to other disulfide oxidoreductases". The Journal of Biological Chemistry. 262 (36): 17313–8. doi:10.1016/S0021-9258(18)45379-3. PMID 3693355.

[pmid3278312-7] Pons G, Raefsky-Estrin C, Carothers DJ, Pepin RA, Javed AA, Jesse BW, et al. (March 1988). "Cloning and cDNA sequence of the dihydrolipoamide dehydrogenase component human alpha-ketoacid dehydrogenase complexes". Proceedings of the National Academy of Sciences of the United States of America. 85 (5): 1422–6. Bibcode:1988PNAS...85.1422P. doi:10.1073/pnas.85.5.1422. PMC 279783. PMID 3278312.

[pmid2055113-8] Scherer SW, Otulakowski G, Robinson BH, Tsui LC (1991). "Localization of the human dihydrolipoamide dehydrogenase gene (DLD) to 7q31----q32". Cytogenetics and Cell Genetics. 56 (3–4): 176–7. doi:10.1159/000133081. hdl:10722/42531. PMID 2055113.

[Babady-9] Babady NE, Pang YP, Elpeleg O, Isaya G (April 2007). "Cryptic proteolytic activity of dihydrolipoamide dehydrogenase". Proceedings of the National Academy of Sciences of the United States of America. 104 (15): 6158–63. Bibcode:2007PNAS..104.6158B. doi:10.1073/pnas.0610618104. PMC 1851069. PMID 17404228.

[Ciszak-10] Ciszak EM, Makal A, Hong YS, Vettaikkorumakankauv AK, Korotchkina LG, Patel MS (January 2006). "How dihydrolipoamide dehydrogenase-binding protein binds dihydrolipoamide dehydrogenase in the human pyruvate dehydrogenase complex". The Journal of Biological Chemistry. 281 (1): 648–55. doi:10.1074/jbc.M507850200. PMID 16263718.

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