Names & Taxonomy

Uniprot ID:
P09622
Entry Name:
DLDH_HUMAN
Status:
reviewed
Protein Names:
Dihydrolipoyl dehydrogenase, mitochondrial (EC 1.8.1.4) (Dihydrolipoamide dehydrogenase) (Glycine cleavage system L protein)
Gene Names:
DLD GCSL LAD PHE3
Gene Names Primary:
DLD
Organism:
Homo sapiens (Human)

Structure

Length:
509
Sequence:
MQSWSRVYCSLAKRGHFNRISHGLQGLSAVPLRTYADQPIDADVTVIGSGPGGYVAAIKAAQLGFKTVCIEKNETLGGTCLNVGCIPSKALLNNSHYYHMAHGKDFASRGIEMSEVRLNLDKMMEQKSTAVKALTGGIAHLFKQNKVVHVNGYGKITGKNQVTATKADGGTQVIDTKNILIATGSEVTPFPGITIDEDTIVSSTGALSLKKVPEKMVVIGAGVIGVELGSVWQRLGADVTAVEFLGHVGGVGIDMEISKNFQRILQKQGFKFKLNTKVTGATKKSDGKIDVSIEAASGGKAEVITCDVLLVCIGRRPFTKNLGLEELGIELDPRGRIPVNTRFQTKIPNIYAIGDVVAGPMLAHKAEDEGIICVEGMAGGAVHIDYNCVPSVIYTHPEVAWVGKSEEQLKEEGIEYKVGKFPFAANSRAKTNADTDGMVKILGQKSTDRVLGAHILGPGAGEMVNEAALALEYGASCEDIARVCHAHPTLSEAFREANLAASFGKSINF
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Mitochondrion matrix.

Function

Function:
Lipoamide dehydrogenase is a component of the glycine cleavage system as well as of the alpha-ketoacid dehydrogenase complexes. Involved in the hyperactivation of spermatazoa during capacitation and in the spermatazoal acrosome reaction.
Catalytic Activity:
Protein N(6)-(dihydrolipoyl)lysine + NAD(+) = protein N(6)-(lipoyl)lysine + NADH.
Cofactor:
COFACTOR: Name=FAD; Xref=ChEBI:CHEBI:57692;
Active Site:
ACT_SITE 487 487 Proton acceptor.
Cross Reference Drug Bank:
DB03147
Gene Ontology Go:
acrosomal matrix
cilium
mitochondrial matrix
mitochondrion
myelin sheath
nucleoplasm
oxoglutarate dehydrogenase complex
pyruvate dehydrogenase complex
dihydrolipoyl dehydrogenase activity
flavin adenine dinucleotide binding
lipoamide binding
NAD binding
2-oxoglutarate metabolic process
aging
branched-chain amino acid catabolic process
cell redox homeostasis
cellular metabolic process
cellular nitrogen compound metabolic process
dihydrolipoamide metabolic process
gastrulation
glyoxylate metabolic process
lipoate metabolic process
lysine catabolic process
mitochondrial acetyl-CoA biosynthetic process from pyruvate
mitochondrial electron transport, NADH to ubiquinone
proteolysis
pyruvate metabolic process
regulation of acetyl-CoA biosynthetic process from pyruvate
regulation of membrane potential
small molecule metabolic process
sperm capacitation
tricarboxylic acid cycle
Gene Ontology Biological Process:
2-oxoglutarate metabolic process
aging
branched-chain amino acid catabolic process
cell redox homeostasis
cellular metabolic process
cellular nitrogen compound metabolic process
dihydrolipoamide metabolic process
gastrulation
glyoxylate metabolic process
lipoate metabolic process
lysine catabolic process
mitochondrial acetyl-CoA biosynthetic process from pyruvate
mitochondrial electron transport, NADH to ubiquinone
proteolysis
pyruvate metabolic process
regulation of acetyl-CoA biosynthetic process from pyruvate
regulation of membrane potential
small molecule metabolic process
sperm capacitation
tricarboxylic acid cycle
Gene Ontology Molecular Function:
dihydrolipoyl dehydrogenase activity
flavin adenine dinucleotide binding
lipoamide binding
NAD binding
Gene Ontology Cellular Component:
acrosomal matrix
cilium
mitochondrial matrix
mitochondrion
myelin sheath
nucleoplasm
oxoglutarate dehydrogenase complex
pyruvate dehydrogenase complex
Keywords:
3D-structure
Acetylation
Alternative splicing
Complete proteome
Disease mutation
Disulfide bond
FAD
Flavoprotein
Mitochondrion
NAD
Oxidoreductase
Phosphoprotein
Polymorphism
Redox-active center
Reference proteome
Transit peptide

Publication

PubMed ID:
3693355 3278312 8406489 14702039 12853948 12690205 15489334 1332063 19608861 21269460 22905912 24275569 25944712 15946682 16263718 16442803 8506365 8968745 9934985