1. Gene
  2. DLST - dihydrolipoamide S-succinyltransferase Gene

DLST - dihydrolipoamide S-succinyltransferase Gene

Homo sapiens

Also known as DLTS; KGD2; PGL7

Gene ID: 1743 | Gene type: protein coding

About DLST

Cytogenetic location: 14q24.3 Genomic coordinates (GRCh38): 14:74,881,916-74,903,743 (from NCBI)

This gene has 16 transcripts (splice variants), 267 orthologues, 3 paralogues and is associated with 2 phenotypes. Ubiquitous expression in kidney (RPKM 40.7), heart (RPKM 35.9) and 25 other tissues.

Summary

This gene encodes a mitochondrial protein that belongs to the 2-oxoacid dehydrogenase family. This protein is one of the three components (the E2 component) of the 2-oxoglutarate dehydrogenase complex that catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Oct 2011]

DLST Products(2)

mRNA Protein Name
NM_001244883.2 NP_001231812.1 dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial isoform 2 precursor
NM_001933.5 NP_001924.2 dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial isoform 1 precursor
Gene Ontology
  • Molecular Function
  • Biological Process
  • Cellular Component
Molecular Function GO Annotation Evidence Reference Source
enables acyltransferase activity IMP
IMP: Inferred from mutant phenotype
30929736 GOA
enables dihydrolipoyllysine-residue succinyltransferase activity IMP
IMP: Inferred from mutant phenotype
30929736 GOA
enables protein binding IPI
IPI: Inferred from physical interaction
16169070 GOA
Biological Process GO Annotation Evidence Reference Source
involved in tricarboxylic acid cycle IMP
IMP: Inferred from mutant phenotype
30929736 GOA
Cellular Component GO Annotation Evidence Reference Source
located in mitochondrion IDA
IDA: Inferred from direct assay
29211711 GOA
located in nucleus IDA
IDA: Inferred from direct assay
29211711 GOA
part of oxoadipate dehydrogenase complex IDA
IDA: Inferred from direct assay
29191460 GOA
part of oxoglutarate dehydrogenase complex IDA
IDA: Inferred from direct assay
29211711 GOA
EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

DLST Protein Structure

Biotin_lipoyl

Biotin_lipoyl: Biotin-requiring enzyme (72 - 143)

2-oxoacid_dh

2-oxoacid_dh: 2-oxoacid dehydrogenases acyltransferase (catalytic domain) (222 - 451)

  • 0
  • 100
  • 200
  • 300
  • 400
  • 453 a.a.
Protein Preferred Names Protein Names

dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial

E2K

DLST Protein-protein interaction Information

Type
Protein Name Protein ID Interactor Interactor Species Interactor ID Detection Method Reference
Intra
DLST P36957 KIAA1683 Homo sapiens A0JP07 32814053
Intra
DLST P36957 KIAA1683 Homo sapiens A0JP07 32814053
Intra
DLST P36957 KIAA1683 Homo sapiens A0JP07 32814053
Intra
DLST P36957 CAMTA2 Homo sapiens O94983-5 32814053
Intra
DLST P36957 CAMTA2 Homo sapiens O94983-5 32814053
Intra
DLST P36957 CAMTA2 Homo sapiens O94983-5 32814053
Intra
DLST P36957 EVA1B Homo sapiens Q9NVM1 32814053
Intra
DLST P36957 EVA1B Homo sapiens Q9NVM1 32814053
Intra
DLST P36957 EVA1B Homo sapiens Q9NVM1 32814053
Intra
DLST P36957 TPX2 Homo sapiens Q9ULW0 32814053
Intra
DLST P36957 TPX2 Homo sapiens Q9ULW0 32814053
Intra
DLST P36957 TPX2 Homo sapiens Q9ULW0 32814053
Intra
DLST P36957 LGALS1 Homo sapiens P09382 32814053
Intra
DLST P36957 LGALS1 Homo sapiens P09382 32814053
Intra
DLST P36957 LGALS1 Homo sapiens P09382 32814053
Intra
DLST P36957 HUWE1 Homo sapiens Q7Z6Z7-2 32814053
Intra
DLST P36957 HUWE1 Homo sapiens Q7Z6Z7-2 32814053
Intra
DLST P36957 HUWE1 Homo sapiens Q7Z6Z7-2 32814053
Intra
DLST P36957 SP6 Homo sapiens Q3SY56 32814053
Intra
DLST P36957 SP6 Homo sapiens Q3SY56 32814053
Intra
DLST P36957 SP6 Homo sapiens Q3SY56 32814053
Intra
DLST P36957 EPCAM Homo sapiens P16422 32814053
Intra
DLST P36957 EPCAM Homo sapiens P16422 32814053
Intra
DLST P36957 EPCAM Homo sapiens P16422 32814053
Intra
DLST P36957 NEK6 Homo sapiens Q9HC98-4 32814053
Intra
DLST P36957 NEK6 Homo sapiens Q9HC98-4 32814053
Intra
DLST P36957 NEK6 Homo sapiens Q9HC98-4 32814053
Intra
DLST P36957 LGALS8 Homo sapiens O00214-2 32814053
Intra
DLST P36957 LGALS8 Homo sapiens O00214-2 32814053
Intra
DLST P36957 LGALS8 Homo sapiens O00214-2 32814053
Intra
DLST P36957 NFATC2IP Homo sapiens Q8NCF5-2 32814053
Intra
DLST P36957 NFATC2IP Homo sapiens Q8NCF5-2 32814053
Intra
DLST P36957 NFATC2IP Homo sapiens Q8NCF5-2 32814053
Intra
DLST P36957 TRNAU1AP Homo sapiens Q9NX07 32814053
Intra
DLST P36957 TRNAU1AP Homo sapiens Q9NX07 32814053
Intra
DLST P36957 TRNAU1AP Homo sapiens Q9NX07 32814053
Intra
DLST P36957 HSD3B1 Homo sapiens P14060 32814053
Intra
DLST P36957 HSD3B1 Homo sapiens P14060 32814053
Intra
DLST P36957 HSD3B1 Homo sapiens P14060 32814053
Intra
DLST P36957 FAHD2A Homo sapiens Q96GK7 32814053
Intra
DLST P36957 FAHD2A Homo sapiens Q96GK7 32814053
Intra
DLST P36957 FAHD2A Homo sapiens Q96GK7 32814053
Intra
DLST P36957 CRELD2 Homo sapiens Q6UXH1-2 32814053
Intra
DLST P36957 CRELD2 Homo sapiens Q6UXH1-2 32814053
Intra
DLST P36957 CRELD2 Homo sapiens Q6UXH1-2 32814053
Intra
DLST P36957 q9bq29_human Homo sapiens Q9BQ29 32814053
Intra
DLST P36957 q9bq29_human Homo sapiens Q9BQ29 32814053
Intra
DLST P36957 q9bq29_human Homo sapiens Q9BQ29 32814053
Intra
DLST P36957 USP12 Homo sapiens O75317 32814053
Intra
DLST P36957 USP12 Homo sapiens O75317 32814053
Intra
DLST P36957 USP12 Homo sapiens O75317 32814053
Intra
DLST P36957 THBS3 Homo sapiens P49746 32814053
Intra
DLST P36957 THBS3 Homo sapiens P49746 32814053
Intra
DLST P36957 THBS3 Homo sapiens P49746 32814053
Intra
DLST P36957 POC1A Homo sapiens Q8NBT0 32814053
Intra
DLST P36957 POC1A Homo sapiens Q8NBT0 32814053
Intra
DLST P36957 POC1A Homo sapiens Q8NBT0 32814053
Intra
DLST P36957 ISY1 Homo sapiens Q9ULR0 32814053
Intra
DLST P36957 ISY1 Homo sapiens Q9ULR0 32814053
Intra
DLST P36957 ISY1 Homo sapiens Q9ULR0 32814053
Intra
DLST P36957 ZHX1-C8orf76 Homo sapiens Q96EF9 32814053
Intra
DLST P36957 ZHX1-C8orf76 Homo sapiens Q96EF9 32814053
Intra
DLST P36957 ZHX1-C8orf76 Homo sapiens Q96EF9 32814053
Intra
DLST P36957 TMX3 Homo sapiens Q96JJ7-2 32814053
Intra
DLST P36957 TMX3 Homo sapiens Q96JJ7-2 32814053
Intra
DLST P36957 TMX3 Homo sapiens Q96JJ7-2 32814053
Intra
DLST P36957 NXPE1 Homo sapiens Q8N323 32814053
Intra
DLST P36957 NXPE1 Homo sapiens Q8N323 32814053
Intra
DLST P36957 NXPE1 Homo sapiens Q8N323 32814053
Intra
DLST P36957 MGC39372 Homo sapiens Q8TB02 32814053
Intra
DLST P36957 MGC39372 Homo sapiens Q8TB02 32814053
Intra
DLST P36957 MGC39372 Homo sapiens Q8TB02 32814053
Intra
DLST P36957 PRAM1 Homo sapiens Q96QH2 32814053
Intra
DLST P36957 PRAM1 Homo sapiens Q96QH2 32814053
Intra
DLST P36957 PRAM1 Homo sapiens Q96QH2 32814053
Intra
DLST P36957 PYGO1 Homo sapiens Q9Y3Y4 32814053
Intra
DLST P36957 PYGO1 Homo sapiens Q9Y3Y4 32814053
Intra
DLST P36957 PYGO1 Homo sapiens Q9Y3Y4 32814053
Intra
DLST P36957 MYNN Homo sapiens Q9NPC7 32814053
Intra
DLST P36957 MYNN Homo sapiens Q9NPC7 32814053
Intra
DLST P36957 MYNN Homo sapiens Q9NPC7 32814053
Intra
DLST P36957 NAP1L1 Homo sapiens P55209 29128334
Intra
DLST P36957 YWHAE Homo sapiens P62258 29128334
Intra
DLST P36957 YWHAE Homo sapiens P62258 29128334
Intra
DLST P36957 PSMD2 Homo sapiens Q13200 32814053
Intra
DLST P36957 PSMD2 Homo sapiens Q13200 32814053
Intra
DLST P36957 PSMD2 Homo sapiens Q13200 32814053
Intra
DLST P36957 RAP1B Homo sapiens P61224 32814053
Intra
DLST P36957 RAP1B Homo sapiens P61224 32814053
Intra
DLST P36957 RAP1B Homo sapiens P61224 32814053
Intra
DLST P36957 RAB3C Homo sapiens Q96E17 32814053
Intra
DLST P36957 RAB3C Homo sapiens Q96E17 32814053
Intra
DLST P36957 RAB3C Homo sapiens Q96E17 32814053
Intra
DLST P36957 CDK1 Homo sapiens P06493 29128334
Intra
DLST P36957 CDK1 Homo sapiens P06493 29128334
Intra
DLST P36957 HTT Homo sapiens P42858 32814053
Intra
DLST P36957 HTT Homo sapiens P42858 32814053
Intra
DLST P36957 HTT Homo sapiens P42858 32814053
Intra
DLST P36957 PIAS4 Homo sapiens Q8N2W9 32814053
Intra
DLST P36957 PIAS4 Homo sapiens Q8N2W9 32814053
Intra
DLST P36957 PIAS4 Homo sapiens Q8N2W9 32814053
Intra
DLST P36957 MAGEA2 Homo sapiens P43356 32814053
Intra
DLST P36957 MAGEA2 Homo sapiens P43356 32814053
Intra
DLST P36957 MAGEA2 Homo sapiens P43356 32814053
Intra
DLST P36957 NLRP3 Homo sapiens Q96P20 32814053
Intra
DLST P36957 NLRP3 Homo sapiens Q96P20 32814053
Intra
DLST P36957 NLRP3 Homo sapiens Q96P20 32814053
Intra
DLST P36957 SPRED2 Homo sapiens Q7Z698 32814053
Intra
DLST P36957 SPRED2 Homo sapiens Q7Z698 32814053
Intra
DLST P36957 SPRED2 Homo sapiens Q7Z698 32814053
Intra
DLST P36957 PSAT1 Homo sapiens Q9Y617 32814053
Intra
DLST P36957 PSAT1 Homo sapiens Q9Y617 32814053
Intra
DLST P36957 PSAT1 Homo sapiens Q9Y617 32814053
Intra
DLST P36957 RBM5 Homo sapiens P52756 32814053
Intra
DLST P36957 RBM5 Homo sapiens P52756 32814053
Intra
DLST P36957 RBM5 Homo sapiens P52756 32814053
Intra
DLST P36957 IDH1 Homo sapiens O75874 32814053
Intra
DLST P36957 IDH1 Homo sapiens O75874 32814053
Intra
DLST P36957 IDH1 Homo sapiens O75874 32814053
Intra
DLST P36957 RAB31 Homo sapiens Q13636 32814053
Intra
DLST P36957 RAB31 Homo sapiens Q13636 32814053
Intra
DLST P36957 RAB31 Homo sapiens Q13636 32814053
Intra
DLST P36957 RBM17 Homo sapiens Q96I25 32814053
Intra
DLST P36957 RBM17 Homo sapiens Q96I25 32814053
Intra
DLST P36957 RBM17 Homo sapiens Q96I25 32814053
Intra
DLST P36957 THAP7 Homo sapiens Q9BT49 32814053
Intra
DLST P36957 THAP7 Homo sapiens Q9BT49 32814053
Intra
DLST P36957 THAP7 Homo sapiens Q9BT49 32814053
Intra
DLST P36957 TDO2 Homo sapiens P48775 32814053
Intra
DLST P36957 TDO2 Homo sapiens P48775 32814053
Intra
DLST P36957 TDO2 Homo sapiens P48775 32814053
Intra
DLST P36957 PSMC4 Homo sapiens P43686 29128334
Intra
DLST P36957 PSMC4 Homo sapiens P43686 32814053
Intra
DLST P36957 PSMC4 Homo sapiens P43686 32814053
Intra
DLST P36957 PSMC4 Homo sapiens P43686 32814053
Intra
DLST P36957 C1orf216 Homo sapiens Q8TAB5 32814053
Intra
DLST P36957 C1orf216 Homo sapiens Q8TAB5 32814053
Intra
DLST P36957 C1orf216 Homo sapiens Q8TAB5 32814053
Intra
DLST P36957 GJA5 Homo sapiens P36382 32814053
Intra
DLST P36957 GJA5 Homo sapiens P36382 32814053
Intra
DLST P36957 GJA5 Homo sapiens P36382 32814053
Intra
DLST P36957 MMAB Homo sapiens Q96EY8 32814053
Intra
DLST P36957 MMAB Homo sapiens Q96EY8 32814053
Intra
DLST P36957 MMAB Homo sapiens Q96EY8 32814053
Intra
DLST P36957 MYBPHL Homo sapiens A2RUH7 32814053
Intra
DLST P36957 MYBPHL Homo sapiens A2RUH7 32814053
Intra
DLST P36957 MYBPHL Homo sapiens A2RUH7 32814053
Intra
DLST P36957 FUT2 Homo sapiens Q10981 32814053
Intra
DLST P36957 FUT2 Homo sapiens Q10981 32814053
Intra
DLST P36957 FUT2 Homo sapiens Q10981 32814053
Intra
DLST P36957 TPGS2 Homo sapiens Q68CL5-3 32814053
Intra
DLST P36957 TPGS2 Homo sapiens Q68CL5-3 32814053
Intra
DLST P36957 TPGS2 Homo sapiens Q68CL5-3 32814053
Intra
DLST P36957 RASSF2 Homo sapiens P50749 32814053
Intra
DLST P36957 RASSF2 Homo sapiens P50749 32814053
Intra
DLST P36957 RASSF2 Homo sapiens P50749 32814053
Cross: Cross-species interaction Intra: Intraspecies interaction

Recombinant DLST Proteins

Cat. No. Product Name Accession Purity
HY-P75709 DLST Protein, Human (sf9, His) P36957 (D68-L453) ≥95%

DLST Antibodies

Cat. No. Product Name Application Reactivity
HY-P81071 DLST Antibody WB, ELISA, IHC-P, IHC-F, ICC/IF Human, Mouse

Related Diseases

Diseases Alias
Paragangliomas 7

PGL7

Hereditary Paraganglioma-Pheochromocytoma Syndromes

Hereditary Pheochromocytoma-Paraganglioma

Hereditary Paraganglioma-Pheochromocytoma

Familial Pheochromocytoma-Paraganglioma

Paragangliomas 2

Paragangliomas 3

Paragangliomas 4

Sdhx-Related Paraganglioma-Pheochromocytoma

Familial Paraganglioma Syndrome

Familial Paraganglioma-Pheochromocytoma Syndromes

Fpgl

Fpgl/Pheo

Paragangliomas 1

Paraganglioma

Maple Syrup Urine Disease

MSUD

Bckd Deficiency

Branched-Chain Ketoaciduria

Branched-Chain Alpha-Keto Acid Dehydrogenase Deficiency

Keto Acid Decarboxylase Deficiency

Maple Syrup Urine Disease, Type Ii

Branched Chain Ketoaciduria

Classic Maple Syrup Urine Disease

Intermittent Maple Syrup Urine Disease

Maple Syrup Urine Disease, Type Ia

Ketoacidaemia

Bckdh Deficiency

Branched-Chain 2-Ketoacid Dehydrogenase Deficiency

Thiamine-Responsive Maple Syrup Urine Disease

Intermediate Maple Syrup Urine Disease

Maple Syrup Urine Disease Type 1a

Maple Syrup Urine Disease Type 1b

Maple Syrup Urine Disease Type 2

Maple Syrup Urine Disease, Type Ib

Dihydrolipoamide Dehydrogenase Deficiency

Branched-Chain Ketoacid Dehydrogenase Deficiency

Maple Syrup Disease

Ketoacidemia

Classic Bckd Deficiency

Classic Msud

Classic Branched-Chain Alpha-Ketoacid Dehydrogenase Deficiency

Classic Branched-Chain Ketoaciduria

Thiamine-Responsive Bckd Deficiency

Thiamine-Responsive Msud

Thiamine-Responsive Branched-Chain Alpha-Ketoacid Dehydrogenase Deficiency

Intermittent Bckd Deficiency

Intermittent Msud

Intermittent Branched-Chain Alpha-Ketoacid Dehydrogenase Deficiency

Maple Syrup Urine Disease 1a

MSUD1A

Maple Syrup Urine Disease Type Ia

Msud Type Ia

Maple Syrup Urine Disease 1b

MSUD1B

Maple Syrup Urine Disease Type Ib

Msud Type Ib

Maple Syrup Urine Disease 2

MSUD2

Maple Syrup Urine Disease Type Ii

Msud Type Ii

Nadh Cytochrome B5 Reductase Deficiency

Lactic Acidosis, Congenital Infantile, Due To Lad Deficiency

Ketonemia

Maple Syrup Urine Disease, Type 1b

Ketoacid Decarboxylase Deficiency

Oxoacid Decarboxylase Deficiency

Branched Chain Ketoacid Dehydrogenase Deficiency

Msud - [Maple-Syrup-Urine Disease]

Ketoaminoacidaemia

Bckd - [Branched-Chain Alpha-Ketoacid Dehydrogenase Deficiency]

Maple-Syrup-Urine Disorder

Maple-Syrup-Urine Syndrome

Hereditary Spastic Paraplegia 51

Autosomal Dominant Spastic Paraplegia 51

Cpsq4

Spastic Quadriplegic Cerebral Palsy 4

Spg51

Spastic Paraplegia 51, Autosomal Recessive

Alzheimer Disease, Familial, 1

Alzheimer Disease

Alzheimer'S Disease

Presenile And Senile Dementia

AD1

Alzheimer Disease, Susceptibility To

Alzheimer Disease, Late-Onset, Susceptibility To

Alzheimer Disease 1, Familial

AD

Familial Alzheimer Disease

Alzheimer Disease, Late-Onset

Alzheimers Dementia

Alzheimer Dementia

Alzheimer Sclerosis

Alzheimer Syndrome

Alzheimer-Type Dementia

Dat

Primary Senile Degenerative Dementia

Sdat

Alzheimer Disease 1

Autosomal Dominant Alzheimer Disease

Early-Onset Alzheimer Disease With Cerebral Amyloid Angiopathy

Late Onset Alzheimer Disease

Alzheimers Disease

Alzheimer Disease, Early-Onset, With Cerebral Amyloid Angiopathy

Late-Onset Alzheimers Disease

Alzheimer'S Disease Pathway Kegg

Dementia Due To Alzheimer'S Disease

Alzheimer Disease Type 1

Alzheimers

Diseases Alias
Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Waldenstroem'S Macroglobulinemia

Waldenstroem'S Macroglobulinemia

Macroglobulinemia Of Waldenstrom

Lymphoplasmacytic Lymphoma With Igm Gammopathy

Lymphoplasmacytic Lymphoma

Orthologs Information

Species Symbol Source ID
Canis familiaris DLST VGNC VGNC:39988
Felis catus DLST VGNC VGNC:61522
Rattus norvegicus DLST RGD RGD:1359615
Mus musculus DLST MGD MGI:1926170
Bos taurus DLST VGNC VGNC:28097
Macaca mulatta DLST VGNC VGNC:71927
Others DLST NCBI