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DLD dimer:2xFAD [mitochondrial matrix]
Stable Identifier
R-HSA-5694020
Type
Complex
Species
Homo sapiens
Compartment
mitochondrial matrix
Locations in the PathwayBrowser
Expand all
Disease (Homo sapiens)
Diseases of metabolism (Homo sapiens)
Diseases of branched-chain amino acid catabolism (Homo sapiens)
Branched-chain ketoacid dehydrogenase kinase deficiency (Homo sapiens)
BCKDK loss-of-function mutations do not phosphorylate BCKDH (Homo sapiens)
BCKDH [mitochondrial matrix] (Homo sapiens)
DLD dimer:2xFAD [mitochondrial matrix] (Homo sapiens)
Maple Syrup Urine Disease (Homo sapiens)
H139Hfs13* PPM1K causes a mild variant of MSUD (Homo sapiens)
H139Hfs13* PPM1K does not dephosphorylate BCKDH (Homo sapiens)
p-BCKDH [mitochondrial matrix] (Homo sapiens)
DLD dimer:2xFAD [mitochondrial matrix] (Homo sapiens)
Loss-of-function mutations in DBT cause MSUD2 (Homo sapiens)
DBT loss-of-function mutants don't synthesize BCAA-CoA (Homo sapiens)
DBT mutant-BCKDH [mitochondrial matrix] (Homo sapiens)
DLD dimer:2xFAD [mitochondrial matrix] (Homo sapiens)
Metabolism (Homo sapiens)
Aerobic respiration and respiratory electron transport (Homo sapiens)
Citric acid cycle (TCA cycle) (Homo sapiens)
OGDH complex synthesizes succinyl-CoA from 2-OG (Homo sapiens)
DLD dimer dehydrogenates dihydrolipoyl (Homo sapiens)
DLD dimer:2xFAD [mitochondrial matrix] (Homo sapiens)
OGDH complex [mitochondrial matrix] (Homo sapiens)
DLD dimer:2xFAD [mitochondrial matrix] (Homo sapiens)
DLST transfers succinyl to CoA (Homo sapiens)
OGDH complex [mitochondrial matrix] (Homo sapiens)
DLD dimer:2xFAD [mitochondrial matrix] (Homo sapiens)
OGDH dimer decarboxylates 2-OG (Homo sapiens)
OGDH complex [mitochondrial matrix] (Homo sapiens)
DLD dimer:2xFAD [mitochondrial matrix] (Homo sapiens)
Pyruvate metabolism (Homo sapiens)
PDH complex synthesizes acetyl-CoA from PYR (Homo sapiens)
DLAT trimer transfers acetyl to CoA (Homo sapiens)
PDH complex [mitochondrial matrix] (Homo sapiens)
DLD dimer:2xFAD [mitochondrial matrix] (Homo sapiens)
DLD dimer dehydrogenates dihydrolipoyl (Homo sapiens)
DLD dimer:2xFAD [mitochondrial matrix] (Homo sapiens)
PDH complex [mitochondrial matrix] (Homo sapiens)
DLD dimer:2xFAD [mitochondrial matrix] (Homo sapiens)
PDH E1 decarboxylates PYR, transferring acetyl to DLAT (Homo sapiens)
PDH complex [mitochondrial matrix] (Homo sapiens)
DLD dimer:2xFAD [mitochondrial matrix] (Homo sapiens)
Regulation of pyruvate metabolism (Homo sapiens)
Regulation of pyruvate dehydrogenase (PDH) complex (Homo sapiens)
PDK isozymes phosphorylate PDHC subunit E1 (Homo sapiens)
PDH complex [mitochondrial matrix] (Homo sapiens)
DLD dimer:2xFAD [mitochondrial matrix] (Homo sapiens)
PDP1,2 dephosphorylate p-lipo-PDH (Homo sapiens)
PDH complex [mitochondrial matrix] (Homo sapiens)
DLD dimer:2xFAD [mitochondrial matrix] (Homo sapiens)
Metabolism of amino acids and derivatives (Homo sapiens)
Branched-chain amino acid catabolism (Homo sapiens)
BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV (Homo sapiens)
BCKDHA:BCKDHB tetramer decarboxylates KIC, KMVA, KIV (Homo sapiens)
BCKDH [mitochondrial matrix] (Homo sapiens)
DLD dimer:2xFAD [mitochondrial matrix] (Homo sapiens)
DBT transfers BCAA to CoA (Homo sapiens)
BCKDH [mitochondrial matrix] (Homo sapiens)
DLD dimer:2xFAD [mitochondrial matrix] (Homo sapiens)
DLD dimer dehydrogenates dihydrolipoyl (Homo sapiens)
BCKDH [mitochondrial matrix] (Homo sapiens)
DLD dimer:2xFAD [mitochondrial matrix] (Homo sapiens)
DLD dimer:2xFAD [mitochondrial matrix] (Homo sapiens)
BCKDK phosphorylates BCKDH (Homo sapiens)
BCKDH [mitochondrial matrix] (Homo sapiens)
DLD dimer:2xFAD [mitochondrial matrix] (Homo sapiens)
p-BCKDH [mitochondrial matrix] (Homo sapiens)
DLD dimer:2xFAD [mitochondrial matrix] (Homo sapiens)
PPM1K dephosphorylates p-BCKDH (Homo sapiens)
BCKDH [mitochondrial matrix] (Homo sapiens)
DLD dimer:2xFAD [mitochondrial matrix] (Homo sapiens)
p-BCKDH [mitochondrial matrix] (Homo sapiens)
DLD dimer:2xFAD [mitochondrial matrix] (Homo sapiens)
Glyoxylate metabolism and glycine degradation (Homo sapiens)
Glycine degradation (Homo sapiens)
DLD dimer:2xFAD oxidises GCSH:DHLL to GCSH:lipoate (Homo sapiens)
DLD dimer:2xFAD [mitochondrial matrix] (Homo sapiens)
OGDH complex [mitochondrial matrix] (Homo sapiens)
DLD dimer:2xFAD [mitochondrial matrix] (Homo sapiens)
Lysine catabolism (Homo sapiens)
OADH complex synthesizes glutaryl-CoA from 2-OA (Homo sapiens)
DHTKD1 dimer decarboxylates 2-OA (Homo sapiens)
OADH complex [mitochondrial matrix] (Homo sapiens)
DLD dimer:2xFAD [mitochondrial matrix] (Homo sapiens)
DLD dimer dehydrogenates dihydrolipoyl (Homo sapiens)
DLD dimer:2xFAD [mitochondrial matrix] (Homo sapiens)
OADH complex [mitochondrial matrix] (Homo sapiens)
DLD dimer:2xFAD [mitochondrial matrix] (Homo sapiens)
DLST transfers glutaryl to CoA (Homo sapiens)
OADH complex [mitochondrial matrix] (Homo sapiens)
DLD dimer:2xFAD [mitochondrial matrix] (Homo sapiens)
Signal Transduction (Homo sapiens)
Signaling by Nuclear Receptors (Homo sapiens)
Signaling by Retinoic Acid (Homo sapiens)
PDK isozymes phosphorylate PDHC subunit E1 (Homo sapiens)
PDH complex [mitochondrial matrix] (Homo sapiens)
DLD dimer:2xFAD [mitochondrial matrix] (Homo sapiens)
Participants
components
x 2
DLD [mitochondrial matrix]
(Homo sapiens)
x 2
FAD [mitochondrial matrix]
Participates
as a component of
DBT mutant-BCKDH [mitochondrial matrix] (Homo sapiens)
OADH complex [mitochondrial matrix] (Homo sapiens)
OGDH complex [mitochondrial matrix] (Homo sapiens)
p-BCKDH [mitochondrial matrix] (Homo sapiens)
BCKDH [mitochondrial matrix] (Homo sapiens)
PDH complex [mitochondrial matrix] (Homo sapiens)
Inferred To
DLD dimer:2xFAD [mitochondrial matrix]
(Bos taurus)
DLD dimer:2xFAD [mitochondrial matrix]
(Caenorhabditis elegans)
DLD dimer:2xFAD [mitochondrial matrix]
(Canis familiaris)
DLD dimer:2xFAD [mitochondrial matrix]
(Danio rerio)
DLD dimer:2xFAD [mitochondrial matrix]
(Dictyostelium discoideum)
DLD dimer:2xFAD [mitochondrial matrix]
(Drosophila melanogaster)
DLD dimer:2xFAD [mitochondrial matrix]
(Gallus gallus)
DLD dimer:2xFAD [mitochondrial matrix]
(Mus musculus)
DLD dimer:2xFAD [mitochondrial matrix]
(Plasmodium falciparum)
DLD dimer:2xFAD [mitochondrial matrix]
(Rattus norvegicus)
DLD dimer:2xFAD [mitochondrial matrix]
(Saccharomyces cerevisiae)
DLD dimer:2xFAD [mitochondrial matrix]
(Schizosaccharomyces pombe)
DLD dimer:2xFAD [mitochondrial matrix]
(Sus scrofa)
DLD dimer:2xFAD [mitochondrial matrix]
(Xenopus tropicalis)
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