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dihydrolipoamide dehydrogenase

Summary

Gene Symbol

Aliases

DLDH
E3
E3 component of pyruvate dehydrogenase complex, 2-oxo-glutarate complex, branched chain keto acid dehydrogenase complex
OGDC-E3

Organism

Homo sapiens (human)

External Links

NCBI Gene

1738

HGNC

2898

KEGG Gene ID

["hsa:1738"]

PubChem

1738

Alliance of Genome Resources

HGNC:2898

JoGo

DLD

TogoVar

DLD

Annotation

Keyword

3D-structure
Acetylation
Alternative splicing
Cytoplasmic vesicle
Disease variant
Disulfide bond
FAD
Flagellum
Mitochondrion
NAD
Nucleotide-binding
Nucleus
Oxidoreductase
Phosphoprotein
Proteomics identification
Redox-active center
Reference proteome
Transit peptide

Proteins

Displaying **all 3** entries
UniProt	Protein Name
P09622	Dihydrolipoamide dehydrogenase Glycine cleavage system L protein
A0A024R713
E9PEX6

Annotation information from UniProt.

Gene Ontology (GO)

Displaying entries **1 - 5** of 19 in total

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GO Term	Evidence Code	PMID
pyruvate decarboxylation to acetyl-CoA	IC
pyruvate decarboxylation to acetyl-CoA	IDA
pyruvate decarboxylation to acetyl-CoA	IEA
pyruvate metabolic process	IBA
tricarboxylic acid cycle	NAS

GO Hierarchy

biological process

cellular process [inference]

metabolic process [inference]

generation of precursor metabolites and energy [inference]

energy derivation by oxidation of organic compounds [inference]

cellular respiration

aerobic respiration [inference]

aerobic electron transport chain [inference]

mitochondrial electron transport, NADH to ubiquinone

electron transport chain [inference]

respiratory electron transport chain [inference]

aerobic electron transport chain [inference]

mitochondrial electron transport, NADH to ubiquinone

sulfur compound metabolic process [inference]

acyl-CoA metabolic process [inference]

acetyl-CoA metabolic process [inference]

acetyl-CoA biosynthetic process [inference]

pyruvate decarboxylation to acetyl-CoA

succinyl-CoA metabolic process [inference]

succinyl-CoA biosynthetic process [inference]

2-oxoglutarate decarboxylation to succinyl-CoA

fatty-acyl-CoA metabolic process [inference]

branched-chain alpha-keto acid decarboxylation to branched-chain acyl-CoA

acyl-CoA biosynthetic process [inference]

acetyl-CoA biosynthetic process [inference]

pyruvate decarboxylation to acetyl-CoA

succinyl-CoA biosynthetic process [inference]

2-oxoglutarate decarboxylation to succinyl-CoA

sulfur compound biosynthetic process [inference]

acyl-CoA biosynthetic process [inference]

acetyl-CoA biosynthetic process [inference]

pyruvate decarboxylation to acetyl-CoA

succinyl-CoA biosynthetic process [inference]

2-oxoglutarate decarboxylation to succinyl-CoA

phosphorus metabolic process [inference]

phosphate-containing compound metabolic process [inference]

nucleoside phosphate metabolic process [inference]

acyl-CoA metabolic process [inference]

acetyl-CoA metabolic process [inference]

acetyl-CoA biosynthetic process [inference]

pyruvate decarboxylation to acetyl-CoA

succinyl-CoA metabolic process [inference]

succinyl-CoA biosynthetic process [inference]

2-oxoglutarate decarboxylation to succinyl-CoA

fatty-acyl-CoA metabolic process [inference]

branched-chain alpha-keto acid decarboxylation to branched-chain acyl-CoA

acyl-CoA biosynthetic process [inference]

acetyl-CoA biosynthetic process [inference]

pyruvate decarboxylation to acetyl-CoA

succinyl-CoA biosynthetic process [inference]

2-oxoglutarate decarboxylation to succinyl-CoA

nucleoside phosphate biosynthetic process [inference]

acyl-CoA biosynthetic process [inference]

acetyl-CoA biosynthetic process [inference]

pyruvate decarboxylation to acetyl-CoA

succinyl-CoA biosynthetic process [inference]

2-oxoglutarate decarboxylation to succinyl-CoA

organophosphate metabolic process [inference]

nucleoside phosphate metabolic process [inference]

acyl-CoA metabolic process [inference]

acetyl-CoA metabolic process [inference]

acetyl-CoA biosynthetic process [inference]

pyruvate decarboxylation to acetyl-CoA

succinyl-CoA metabolic process [inference]

succinyl-CoA biosynthetic process [inference]

2-oxoglutarate decarboxylation to succinyl-CoA

fatty-acyl-CoA metabolic process [inference]

branched-chain alpha-keto acid decarboxylation to branched-chain acyl-CoA

acyl-CoA biosynthetic process [inference]

acetyl-CoA biosynthetic process [inference]

pyruvate decarboxylation to acetyl-CoA

succinyl-CoA biosynthetic process [inference]

2-oxoglutarate decarboxylation to succinyl-CoA

nucleoside phosphate biosynthetic process [inference]

acyl-CoA biosynthetic process [inference]

acetyl-CoA biosynthetic process [inference]

pyruvate decarboxylation to acetyl-CoA

succinyl-CoA biosynthetic process [inference]

2-oxoglutarate decarboxylation to succinyl-CoA

organophosphate biosynthetic process [inference]

nucleoside phosphate biosynthetic process [inference]

acyl-CoA biosynthetic process [inference]

acetyl-CoA biosynthetic process [inference]

pyruvate decarboxylation to acetyl-CoA

succinyl-CoA biosynthetic process [inference]

2-oxoglutarate decarboxylation to succinyl-CoA

catabolic process [inference]

amino acid catabolic process [inference]

branched-chain amino acid catabolic process

alpha-amino acid catabolic process [inference]

L-amino acid catabolic process [inference]

L-lysine catabolic process

small molecule catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

branched-chain amino acid catabolic process

proteinogenic amino acid catabolic process [inference]

L-lysine catabolic process

alpha-amino acid catabolic process [inference]

L-amino acid catabolic process [inference]

L-lysine catabolic process

biosynthetic process [inference]

nucleobase-containing compound biosynthetic process [inference]

nucleoside phosphate biosynthetic process [inference]

acyl-CoA biosynthetic process [inference]

acetyl-CoA biosynthetic process [inference]

pyruvate decarboxylation to acetyl-CoA

succinyl-CoA biosynthetic process [inference]

2-oxoglutarate decarboxylation to succinyl-CoA

sulfur compound biosynthetic process [inference]

acyl-CoA biosynthetic process [inference]

acetyl-CoA biosynthetic process [inference]

pyruvate decarboxylation to acetyl-CoA

succinyl-CoA biosynthetic process [inference]

2-oxoglutarate decarboxylation to succinyl-CoA

purine-containing compound biosynthetic process [inference]

acyl-CoA biosynthetic process [inference]

acetyl-CoA biosynthetic process [inference]

pyruvate decarboxylation to acetyl-CoA

succinyl-CoA biosynthetic process [inference]

2-oxoglutarate decarboxylation to succinyl-CoA

organophosphate biosynthetic process [inference]

nucleoside phosphate biosynthetic process [inference]

acyl-CoA biosynthetic process [inference]

acetyl-CoA biosynthetic process [inference]

pyruvate decarboxylation to acetyl-CoA

succinyl-CoA biosynthetic process [inference]

2-oxoglutarate decarboxylation to succinyl-CoA

macromolecule metabolic process [inference]

protein metabolic process [inference]

proteolysis

primary metabolic process [inference]

tricarboxylic acid cycle

nucleobase-containing compound metabolic process [inference]

nucleobase-containing compound biosynthetic process [inference]

nucleoside phosphate biosynthetic process [inference]

acyl-CoA biosynthetic process [inference]

acetyl-CoA biosynthetic process [inference]

pyruvate decarboxylation to acetyl-CoA

succinyl-CoA biosynthetic process [inference]

2-oxoglutarate decarboxylation to succinyl-CoA

nucleobase-containing small molecule metabolic process [inference]

nucleoside phosphate metabolic process [inference]

acyl-CoA metabolic process [inference]

acetyl-CoA metabolic process [inference]

acetyl-CoA biosynthetic process [inference]

pyruvate decarboxylation to acetyl-CoA

succinyl-CoA metabolic process [inference]

succinyl-CoA biosynthetic process [inference]

2-oxoglutarate decarboxylation to succinyl-CoA

fatty-acyl-CoA metabolic process [inference]

branched-chain alpha-keto acid decarboxylation to branched-chain acyl-CoA

acyl-CoA biosynthetic process [inference]

acetyl-CoA biosynthetic process [inference]

pyruvate decarboxylation to acetyl-CoA

succinyl-CoA biosynthetic process [inference]

2-oxoglutarate decarboxylation to succinyl-CoA

nucleoside phosphate biosynthetic process [inference]

acyl-CoA biosynthetic process [inference]

acetyl-CoA biosynthetic process [inference]

pyruvate decarboxylation to acetyl-CoA

succinyl-CoA biosynthetic process [inference]

2-oxoglutarate decarboxylation to succinyl-CoA

amino acid metabolic process [inference]

amino acid catabolic process [inference]

branched-chain amino acid catabolic process

alpha-amino acid catabolic process [inference]

L-amino acid catabolic process [inference]

L-lysine catabolic process

branched-chain amino acid metabolic process [inference]

branched-chain amino acid catabolic process

proteinogenic amino acid metabolic process [inference]

proteinogenic amino acid catabolic process [inference]

L-lysine catabolic process

alpha-amino acid metabolic process [inference]

L-amino acid metabolic process [inference]

L-amino acid catabolic process [inference]

L-lysine catabolic process

alpha-amino acid catabolic process [inference]

L-amino acid catabolic process [inference]

L-lysine catabolic process

lipid metabolic process [inference]

fatty acid derivative metabolic process [inference]

fatty-acyl-CoA metabolic process [inference]

branched-chain alpha-keto acid decarboxylation to branched-chain acyl-CoA

protein metabolic process [inference]

proteolysis

small molecule metabolic process [inference]

organic acid metabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

branched-chain amino acid catabolic process

proteinogenic amino acid catabolic process [inference]

L-lysine catabolic process

alpha-amino acid catabolic process [inference]

L-amino acid catabolic process [inference]

L-lysine catabolic process

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

branched-chain amino acid metabolic process [inference]

branched-chain amino acid catabolic process

monocarboxylic acid metabolic process [inference]

pyruvate metabolic process

pyruvate decarboxylation to acetyl-CoA

dicarboxylic acid metabolic process [inference]

2-oxoglutarate metabolic process

2-oxoglutarate decarboxylation to succinyl-CoA

carboxylic acid catabolic process [inference]

branched-chain amino acid catabolic process

proteinogenic amino acid catabolic process [inference]

L-lysine catabolic process

alpha-amino acid catabolic process [inference]

L-amino acid catabolic process [inference]

L-lysine catabolic process

branched-chain alpha-keto acid decarboxylation to branched-chain acyl-CoA

proteinogenic amino acid metabolic process [inference]

proteinogenic amino acid catabolic process [inference]

L-lysine catabolic process

alpha-amino acid metabolic process [inference]

L-amino acid metabolic process [inference]

L-amino acid catabolic process [inference]

L-lysine catabolic process

alpha-amino acid catabolic process [inference]

L-amino acid catabolic process [inference]

L-lysine catabolic process

small molecule catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

branched-chain amino acid catabolic process

proteinogenic amino acid catabolic process [inference]

L-lysine catabolic process

alpha-amino acid catabolic process [inference]

L-amino acid catabolic process [inference]

L-lysine catabolic process

nucleobase-containing small molecule metabolic process [inference]

nucleoside phosphate metabolic process [inference]

acyl-CoA metabolic process [inference]

acetyl-CoA metabolic process [inference]

acetyl-CoA biosynthetic process [inference]

pyruvate decarboxylation to acetyl-CoA

succinyl-CoA metabolic process [inference]

succinyl-CoA biosynthetic process [inference]

2-oxoglutarate decarboxylation to succinyl-CoA

fatty-acyl-CoA metabolic process [inference]

branched-chain alpha-keto acid decarboxylation to branched-chain acyl-CoA

acyl-CoA biosynthetic process [inference]

acetyl-CoA biosynthetic process [inference]

pyruvate decarboxylation to acetyl-CoA

succinyl-CoA biosynthetic process [inference]

2-oxoglutarate decarboxylation to succinyl-CoA

nucleoside phosphate biosynthetic process [inference]

acyl-CoA biosynthetic process [inference]

acetyl-CoA biosynthetic process [inference]

pyruvate decarboxylation to acetyl-CoA

succinyl-CoA biosynthetic process [inference]

2-oxoglutarate decarboxylation to succinyl-CoA

purine-containing compound metabolic process [inference]

acyl-CoA metabolic process [inference]

acetyl-CoA metabolic process [inference]

acetyl-CoA biosynthetic process [inference]

pyruvate decarboxylation to acetyl-CoA

succinyl-CoA metabolic process [inference]

succinyl-CoA biosynthetic process [inference]

2-oxoglutarate decarboxylation to succinyl-CoA

fatty-acyl-CoA metabolic process [inference]

branched-chain alpha-keto acid decarboxylation to branched-chain acyl-CoA

acyl-CoA biosynthetic process [inference]

acetyl-CoA biosynthetic process [inference]

pyruvate decarboxylation to acetyl-CoA

succinyl-CoA biosynthetic process [inference]

2-oxoglutarate decarboxylation to succinyl-CoA

purine-containing compound biosynthetic process [inference]

acyl-CoA biosynthetic process [inference]

acetyl-CoA biosynthetic process [inference]

pyruvate decarboxylation to acetyl-CoA

succinyl-CoA biosynthetic process [inference]

2-oxoglutarate decarboxylation to succinyl-CoA

cellular process involved in reproduction in multicellular organism [inference]

sperm capacitation

cellular developmental process [inference]

cell maturation [inference]

sperm capacitation

reproductive process [inference]

developmental process involved in reproduction [inference]

sperm capacitation

multicellular organismal reproductive process [inference]

cellular process involved in reproduction in multicellular organism [inference]

sperm capacitation

developmental process [inference]

developmental process involved in reproduction [inference]

sperm capacitation

anatomical structure morphogenesis [inference]

embryonic morphogenesis [inference]

gastrulation

developmental maturation [inference]

anatomical structure maturation [inference]

cell maturation [inference]

sperm capacitation

cellular developmental process [inference]

cell maturation [inference]

sperm capacitation

biological regulation [inference]

regulation of biological quality [inference]

regulation of membrane potential

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GO Term	Evidence Code	PMID
acrosomal vesicle	IEA
nucleus	IDA
nucleus	IEA
nucleoplasm	IDA
mitochondrion	NAS

GO Hierarchy

cellular component

protein-containing complex [inference]

catalytic complex [inference]

tricarboxylic acid cycle heteromeric enzyme complex [inference]

oxoglutarate dehydrogenase complex

oxidoreductase complex [inference]

alpha-ketoacid dehydrogenase complex [inference]

transferase complex [inference]

acetyltransferase complex [inference]

pyruvate dehydrogenase complex

cellular anatomical structure [inference]

nucleoplasm

membrane-enclosed lumen [inference]

organelle lumen [inference]

intracellular organelle lumen [inference]

mitochondrial matrix

cell projection [inference]

plasma membrane bounded cell projection [inference]

cilium

motile cilium

acrosomal matrix

organelle [inference]

membrane-bounded organelle [inference]

cilium

motile cilium

vesicle [inference]

intracellular vesicle [inference]

cytoplasmic vesicle [inference]

secretory vesicle [inference]

secretory granule [inference]

acrosomal vesicle

intracellular membrane-bounded organelle [inference]

intracellular vesicle [inference]

cytoplasmic vesicle [inference]

secretory vesicle [inference]

secretory granule [inference]

acrosomal vesicle

intracellular organelle [inference]

intracellular membrane-bounded organelle [inference]

intracellular vesicle [inference]

cytoplasmic vesicle [inference]

secretory vesicle [inference]

secretory granule [inference]

acrosomal vesicle

Displaying entries **1 - 5** of 11 in total

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GO Term	Evidence Code	PMID
dihydrolipoyl dehydrogenase (NADH) activity	EXP
dihydrolipoyl dehydrogenase (NADH) activity	TAS
dihydrolipoyl dehydrogenase (NADH) activity	IDA
dihydrolipoyl dehydrogenase (NADH) activity	IMP
dihydrolipoyl dehydrogenase (NADH) activity	IBA

GO Hierarchy

molecular function

catalytic activity [inference]

oxidoreductase activity

oxidoreductase activity, acting on a sulfur group of donors [inference]

oxidoreductase activity, acting on a sulfur group of donors, NAD(P) as acceptor

dihydrolipoyl dehydrogenase (NADH) activity

binding [inference]

protein binding

small molecule binding [inference]

ion binding [inference]

anion binding [inference]

flavin adenine dinucleotide binding

heterocyclic compound binding [inference]

nucleotide binding [inference]

flavin adenine dinucleotide binding

nucleoside phosphate binding [inference]

nucleotide binding [inference]

flavin adenine dinucleotide binding

Gene Ontology information from NCBI Gene and UniProt.

OrthoDB (Group)

Group level

Eukaryota

Group Name

Glutathione

Functional Category

C: Energy production and conversion
E: Amino acid transport and metabolism
T: Signal transduction mechanisms

Displaying **all 4** entries
Gene Ontology
cellular response to oxidative stress
flavin adenine dinucleotide binding
nucleotide binding
oxidoreductase activity

Displaying **all 6** entries
InterPro
FAD/NAD(P)-binding domain superfamily
FAD/NAD(P)-binding domain
FAD/NAD-linked reductase, dimerisation domain superfamily
Pyridine nucleotide-disulphide oxidoreductase, class I, active site
Pyridine nucleotide-disulphide oxidoreductase, class I
Pyridine nucleotide-disulphide oxidoreductase, dimerisation domain

Human Protein Atlas

ENSG00000091140

Tissue with high expression from Human Protein Atlas. Tissues that are highly expressed are highlighted.

Data available from v23.0.proteinatlas.org. Image credit: Human Protein Atlas

KEGG BRITE Database

Orthology

K00382

Name

dihydrolipoyl dehydrogenase [EC:1.8.1.4]

References

Rhea

RHEA:15045

NAD⁺

H⁺ + NADH

Enzyme

1.8.1.4

PMID

RHEA:33059

(R)-dihydrolipoamide + NAD⁺

H⁺ + NADH + (R)-lipoamide

Enzyme

1.8.1.4

PMID

13767908

Disease

Disease Ontology

Displaying 1 entry
DO ID	Disease Name	Source
DOID:9269	maple syrup urine disease	Alliance of Genome Resources

The Human Phenotype Ontology

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HPO ID	HPO Term
HP:0000007	Autosomal recessive inheritance
HP:0000252	Microcephaly
HP:0000708	Atypical behavior
HP:0001250	Seizure
HP:0001251	Ataxia
HP:0001252	Hypotonia
HP:0001254	Lethargy
HP:0001257	Spasticity
HP:0001263	Global developmental delay
HP:0001290	Generalized hypotonia

Displaying **all 2** entries
Disease ID	Disease Name
OMIM:246900	pyruvate dehydrogenase E3 deficiency
ORPHA:2394	pyruvate dehydrogenase E3 deficiency

PubChem Disease

GHR Health Conditions

MedGen Diseases

Pyruvate dehydrogenase E3 deficiency

OMIM Phenotypes

DIHYDROLIPOAMIDE DEHYDROGENASE DEFICIENCY; DLDD

KEGG Diseases

Gene-Disease-Co-Occurrences-in-Literature (PubChem)

LIPID MAPS Gene / Proteome Database

LMPD ID: LMP001594
Gene Name: dihydrolipoamide dehydrogenase

GlyTouCan

GlyComb

GlycoPOST

UniCarb-DR

LM-GlycoRepo

All Resources

Other Information

Tools

Search Tools

Guidelines

Summary

External Links

Annotation

OrthoDB (Group)

KEGG BRITE Database

PubChem Disease

LIPID MAPS Gene / Proteome Database

International Collaboration

Acknowledgements