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Identification
HMDB Protein ID HMDBP00939
Secondary Accession Numbers
  • 6227
Name Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial
Synonyms
  1. 2-oxoglutarate dehydrogenase complex component E2
  2. Dihydrolipoamide succinyltransferase component of 2-oxoglutarate dehydrogenase complex
  3. E2K
  4. OGDC-E2
Gene Name DLST
Protein Type Enzyme
Biological Properties
General Function Involved in acyltransferase activity
Specific Function The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). It contains multiple copies of 3 enzymatic components: 2-oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3).
Pathways
  • 2-aminoadipic 2-oxoadipic aciduria
  • 2-ketoglutarate dehydrogenase complex deficiency
  • Citrate cycle (TCA cycle)
  • Citric Acid Cycle
  • Congenital lactic acidosis
  • Fumarase deficiency
  • Glutaminolysis and Cancer
  • Glutaric Aciduria Type I
  • Hyperlysinemia I, Familial
  • Hyperlysinemia II or Saccharopinuria
  • L-lysine degradation via saccharopine pathway
  • Lysine Degradation
  • Lysine degradation
  • Mitochondrial complex II deficiency
  • Pyridoxine dependency with seizures
  • Pyruvate dehydrogenase deficiency (E2)
  • Pyruvate dehydrogenase deficiency (E3)
  • Saccharopinuria/Hyperlysinemia II
  • The oncogenic action of 2-hydroxyglutarate
  • The oncogenic action of D-2-hydroxyglutarate in Hydroxygluaricaciduria
  • The Oncogenic Action of Fumarate
  • The oncogenic action of L-2-hydroxyglutarate in Hydroxygluaricaciduria
  • The Oncogenic Action of Succinate
  • Warburg Effect
Reactions
Succinyl-CoA + enzyme N(6)-(dihydrolipoyl)lysine → Coenzyme A + enzyme N(6)-(S-succinyldihydrolipoyl)lysine details
Succinyl-CoA + Enzyme N6-(dihydrolipoyl)lysine → Coenzyme A + [Dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine details
Glutaryl-CoA + Enzyme N6-(dihydrolipoyl)lysine → Coenzyme A + [Dihydrolipoyllysine-residue succinyltransferase] S-glutaryldihydrolipoyllysine details
GO Classification
Biological Process
NADH metabolic process
cellular nitrogen compound metabolic process
2-oxoglutarate metabolic process
L-lysine catabolic process to acetyl-CoA via saccharopine
lysine catabolic process
tricarboxylic acid cycle
Cellular Component
mitochondrial matrix
plasma membrane
nucleus
oxoglutarate dehydrogenase complex
Component
oxoglutarate dehydrogenase complex
macromolecular complex
protein complex
Function
catalytic activity
transferase activity
transferase activity, transferring acyl groups
transferase activity, transferring acyl groups other than amino-acyl groups
acyltransferase activity
s-acyltransferase activity
s-succinyltransferase activity
dihydrolipoyllysine-residue succinyltransferase activity
Molecular Function
dihydrolipoyllysine-residue succinyltransferase activity
Process
metabolic process
cellular metabolic process
cofactor metabolic process
coenzyme metabolic process
acetyl-coa metabolic process
acetyl-coa catabolic process
tricarboxylic acid cycle
Cellular Location
  1. Mitochondrion
Gene Properties
Chromosome Location 14
Locus 14q24.3
SNPs DLST
Gene Sequence
>1362 bp
ATGCTGTCCCGATCCCGCTGTGTGTCTCGGGCGTTCAGCCGCTCGCTCTCCGCCTTCCAG
AAGGGGAACTGCCCTCTAGGGAGACGTTCCCTGCCTGGGGTCTCCTTATGCCAGGGACCA
GGTTACCCTAACAGCAGGAAGGTTGTCATTAACAACAGTGTCTTCAGTGTTCGCTTTTTC
AGAACTACAGCTGTATGCAAGGATGACTTGGTTACAGTCAAAACCCCAGCGTTTGCAGAA
TCTGTCACAGAGGGAGATGTCAGGTGGGAGAAAGCTGTTGGAGACACAGTTGCAGAAGAT
GAAGTGGTTTGTGAGATTGAAACTGACAAGACATCTGTGCAGGTTCCATCACCAGCAAAT
GGCGTGATTGAAGCTCTTTTGGTACCTGATGGGGGAAAAGTCGAAGGAGGCACTCCACTT
TTCACACTCAGGAAAACTGGTGCTGCTCCTGCTAAGGCCAAGCCGGCTGAAGCTCCTGCT
GCTGCAGCCCCAAAAGCAGAACCTACAGCAGCGGCAGTTCCTCCCCCTGCAGCACCCATA
CCCACTCAGATGCCACCGGTGCCCTCGCCCTCACAGCCTCCTTCTGGCAAACCTGTGTCT
GCAGTAAAACCCACTGTTGCCCCACCACTAGCTGAGCCAGGAGCTGGCAAAGGTCTGCGT
TCAGAACATCGGGAGAAAATGAACAGGATGCGGCAGCGCATTGCTCAGCGTCTGAAGGAG
GCCCAGAATACATGTGCAATGCTGACAACTTTTAATGAGATTGACATGAGTAACATCCAG
GAGATGAGGGCTCGGCACAAAGAGGCTTTTTTGAAGAAACATAACCTCAAACTAGGCTTC
ATGTCGGCATTTGTGAAGGCCTCAGCCTTTGCCTTGCAGGAACAGCCTGTTGTAAATGCA
GTGATTGACGACACAACCAAAGAGGTGGTGTATAGGGATTATATTGACATCAGTGTTGCA
GTGGCCACCCCACGGGGTCTGGTGGTTCCAGTCATCAGGAATGTGGAAGCTATGAATTTT
GCAGATATTGAACGGACCATCACTGAACTGGGAGAGAAGGCCCGAAAGAATGAACTTGCC
ATTGAAGATATGGATGGCGGTACCTTCACCATTAGCAATGGAGGCGTTTTTGGCTCGCTC
TTTGGAACACCCATTATCAACCCCCCTCAGTCTGCCATCCTGGGGATGCATGGCATCTTT
GACAGGCCAGTGGCTATAGGAGGCAAGGTAGAGGTGCGGCCCATGATGTACGTGGCACTG
ACCTATGATCACCGGCTGATTGATGGCAGAGAGGCTGTGACTTTCCTCCGCAAAATCAAG
GCAGCGGTAGAGGATCCCAGAGTCCTCCTCCTGGATCTTTAG
Protein Properties
Number of Residues 453
Molecular Weight 48754.87
Theoretical pI 8.955
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial
MLSRSRCVSRAFSRSLSAFQKGNCPLGRRSLPGVSLCQGPGYPNSRKVVINNSVFSVRFF
RTTAVCKDDLVTVKTPAFAESVTEGDVRWEKAVGDTVAEDEVVCEIETDKTSVQVPSPAN
GVIEALLVPDGGKVEGGTPLFTLRKTGAAPAKAKPAEAPAAAAPKAEPTAAAVPPPAAPI
PTQMPPVPSPSQPPSGKPVSAVKPTVAPPLAEAGAGKGLRSEHREKMNRMRQRIAQRLKE
AQNTCAMLTTFNEIDMSNIQEMRARHKEAFLKKHNLKLGFMSAFVKASAFALQEQPVVNA
VIDDTTKEVVYRDYIDISVAVATPRGLVVPVIRNVEAMNFADIERTITELGEKARKNELA
IEDMDGGTFTISNGGVFGSLFGTPIINPPQSAILGMHGIFDRPVAIGGKVEVRPMMYVAL
TYDHRLIDGREAVTFLRKIKAAVEDPRVLLLDL
GenBank ID Protein 4809336
UniProtKB/Swiss-Prot ID P36957
UniProtKB/Swiss-Prot Entry Name ODO2_HUMAN
PDB IDs Not Available
GenBank Gene ID AC006530
GeneCard ID DLST
GenAtlas ID DLST
HGNC ID HGNC:2911
References
General References
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