DLST

DLST
Identifiers
Aliases	DLST , DLTS, dihydrolipoamide S-succinyltransferase, PGL7, KGD2
External IDs	OMIM: 126063 MGI: 1926170 HomoloGene: 1456 GeneCards: DLST
Gene location (Human)
Chr.	Chromosome 14 (human)
End	74,903,743 bp
Gene location (Mouse)
Chr.	Chromosome 12 (mouse)
End	85,181,619 bp
RNA expression pattern
	Top expressed in
	left ventricle; ; gastrocnemius muscle; ; left adrenal gland; ; secondary oocyte; ; rectum; ; skin of abdomen; ; gastric mucosa; ; transverse colon; ; canal of the cervix; ; body of stomach;
	Top expressed in
	right ventricle; ; digastric muscle; ; myocardium of ventricle; ; thoracic diaphragm; ; brown adipose tissue; ; soleus muscle; ; cardiac muscles; ; sternocleidomastoid muscle; ; vastus lateralis muscle; ; skeletal muscle tissue;
	More reference expression data
	More reference expression data
Gene ontology
Molecular function	transferase activity ; dihydrolipoyllysine-residue succinyltransferase activity ; acyltransferase activity ; protein binding ;
Cellular component	oxoglutarate dehydrogenase complex ; membrane ; myelin sheath ; mitochondrial matrix ; mitochondrion ; extracellular exosome ; nucleus ;
Biological process	tricarboxylic acid cycle ; lysine catabolic process ; generation of precursor metabolites and energy ; L-lysine catabolic process to acetyl-CoA via saccharopine ; metabolism ; cellular nitrogen compound metabolic process ; 2-oxoglutarate metabolic process ; succinyl-CoA metabolic process ; histone succinylation ;
	Sources:Amigo / QuickGO
Orthologs
	1743
	78920
	ENSG00000119689
	ENSMUSG00000004789
	P36957
	Q9D2G2
	NM_001244883 ; NM_001933
	NM_030225
	NP_001231812 ; NP_001924
	NP_084501
	Wikidata
View/Edit Human	View/Edit Mouse

Last updated November 21, 2023

Dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex, mitochondrial is an enzyme that in humans is encoded by the DLST gene.^[5]^[6]

Interactive pathway map

Click on genes, proteins and metabolites below to link to respective articles.^{[§ 1]}

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|alt=TCACycle_WP78 edit]]

TCACycle_WP78 edit

↑ The interactive pathway map can be edited at WikiPathways: "TCACycle_WP78".

Related Research Articles

α-Ketoglutaric acid is a keto acid.

The oxoglutarate dehydrogenase complex (OGDC) or α-ketoglutarate dehydrogenase complex is an enzyme complex, most commonly known for its role in the citric acid cycle.

The branched-chain α-ketoacid dehydrogenase complex is a multi-subunit complex of enzymes that is found on the mitochondrial inner membrane. This enzyme complex catalyzes the oxidative decarboxylation of branched, short-chain alpha-ketoacids. BCKDC is a member of the mitochondrial α-ketoacid dehydrogenase complex family comprising pyruvate dehydrogenase and alpha-ketoglutarate dehydrogenase, key enzymes that function in the Krebs cycle.

Succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial (SDHB) also known as iron-sulfur subunit of complex II (Ip) is a protein that in humans is encoded by the SDHB gene.

Dihydrolipoyl transacetylase is an enzyme component of the multienzyme pyruvate dehydrogenase complex. The pyruvate dehydrogenase complex is responsible for the pyruvate decarboxylation step that links glycolysis to the citric acid cycle. This involves the transformation of pyruvate from glycolysis into acetyl-CoA which is then used in the citric acid cycle to carry out cellular respiration.

Dihydrolipoamide dehydrogenase (DLD), also known as dihydrolipoyl dehydrogenase, mitochondrial, is an enzyme that in humans is encoded by the DLD gene. DLD is a flavoprotein enzyme that oxidizes dihydrolipoamide to lipoamide.

Glycine decarboxylase also known as glycine cleavage system P protein or glycine dehydrogenase is an enzyme that in humans is encoded by the GLDC gene.

Egl nine homolog 2 is a protein that in humans is encoded by the EGLN2 gene. ELGN2 is an alpha-ketoglutarate-dependent hydroxylase, a superfamily of non-haem iron-containing proteins.

A 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial is an enzyme that in humans is encoded by the BCKDHA gene.

Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial is an enzyme that in humans is encoded by the DBT gene.

Phosphorylase b kinase gamma catalytic chain, skeletal muscle isoform is an enzyme that in humans is encoded by the PHKG1 gene.

Alpha-taxilin also known as interleukin-14 (IL-14) or high molecular weight B-cell growth factor (HMW-BCGF) is a protein that in humans is encoded by the TXLNA gene.

2-Oxoisovalerate dehydrogenase subunit beta, mitochondrial is an enzyme that in humans is encoded by the BCKDHB gene.

Isocitrate dehydrogenase [NADP], mitochondrial is an enzyme that in humans is encoded by the IDH2 gene.

Isocitrate dehydrogenase [NAD] subunit gamma, mitochondrial is an enzyme that in humans is encoded by the IDH3G gene.

Isocitrate dehydrogenase [NAD] subunit beta, mitochondrial is an enzyme that in humans is encoded by the IDH3B gene.

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 is an enzyme that in humans is encoded by the NDUFA5 gene. The NDUFA5 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain.

NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 is an enzyme that in humans is encoded by the NDUFA8 gene. The NDUFA8 protein is a subunit of NADH dehydrogenase (ubiquinone), which is located in the mitochondrial inner membrane and is the largest of the five complexes of the electron transport chain.

Alpha-ketoglutarate dehydrogenase also known as 2-oxoglutarate dehydrogenase E1 component, mitochondrial is an enzyme that in humans is encoded by the OGDH gene.

Pyruvate dehydrogenase (lipoamide) alpha 2, also known as pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial or PDHE1-A type II, is an enzyme that in humans is encoded by the PDHA2 gene.

References

1 2 3 GRCh38: Ensembl release 89: ENSG00000119689 - Ensembl, May 2017
1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000004789 - Ensembl, May 2017
↑ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
↑ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
↑ Ali G, Wasco W, Cai X, Szabo P, Sheu KF, Cooper AJ, Gaston SM, Gusella JF, Tanzi RE, Blass JP (Jul 1994). "Isolation, characterization, and mapping of gene encoding dihydrolipoyl succinyltransferase (E2k) of human alpha-ketoglutarate dehydrogenase complex". Somat Cell Mol Genet. 20 (2): 99–105. doi:10.1007/BF02290679. PMID 8009371. S2CID 43092605.
↑ "Entrez Gene: DLST dihydrolipoamide S-succinyltransferase (E2 component of 2-oxo-glutarate complex)".

Chuang DT, Fisher CW, Lau KS, et al. (1991). "Maple syrup urine disease: domain structure, mutations and exon skipping in the dihydrolipoyl transacylase (E2) component of the branched-chain alpha-keto acid dehydrogenase complex". Mol. Biol. Med. 8 (1): 49–63. PMID 1943690.
Fregeau DR, Prindiville T, Coppel RL, et al. (1990). "Inhibition of alpha-ketoglutarate dehydrogenase activity by a distinct population of autoantibodies recognizing dihydrolipoamide succinyltransferase in primary biliary cirrhosis". Hepatology. 11 (6): 975–81. doi: 10.1002/hep.1840110611 . PMID 2365294. S2CID 83760368.
Ono K, Hakozaki M, Kimura A, Kochi H (1987). "Purification, resolution, and reconstitution of rat liver branched-chain alpha-keto acid dehydrogenase complex". J. Biochem. 101 (1): 19–27. doi: 10.1093/oxfordjournals.jbchem.a121891 . PMID 3571202.
Kochi H, Seino H, Ono K (1986). "Inhibition of glycine oxidation by pyruvate, alpha-ketoglutarate, and branched-chain alpha-keto acids in rat liver mitochondria: presence of interaction between the glycine cleavage system and alpha-keto acid dehydrogenase complexes". Arch. Biochem. Biophys. 249 (2): 263–72. doi:10.1016/0003-9861(86)90002-0. PMID 3753002.
Nakano K, Takase C, Sakamoto T, et al. (1994). "Isolation, characterization and structural organization of the gene and pseudogene for the dihydrolipoamide succinyltransferase component of the human 2-oxoglutarate dehydrogenase complex". Eur. J. Biochem. 224 (1): 179–89. doi: 10.1111/j.1432-1033.1994.tb20010.x . PMID 8076640.
Nakano K, Takase C, Sakamoto T, et al. (1993). "An unspliced cDNA for human dihydrolipoamide succinyltransferase: characterization and mapping of the gene to chromosome 14q24.2-q24.3". Biochem. Biophys. Res. Commun. 196 (2): 527–33. doi:10.1006/bbrc.1993.2282. PMID 8240324.
Nakano K, Matuda S, Sakamoto T, et al. (1994). "Human dihydrolipoamide succinyltransferase: cDNA cloning and localization on chromosome 14q24.2-q24.3". Biochim. Biophys. Acta. 1216 (3): 360–8. doi:10.1016/0167-4781(93)90002-u. PMID 8268217.
Cruts M, Backhovens H, Van Gassen G, et al. (1996). "Mutation analysis of the chromosome 14q24.3 dihydrolipoyl succinyltransferase (DLST) gene in patients with early-onset Alzheimer disease". Neurosci. Lett. 199 (1): 73–7. doi:10.1016/0304-3940(95)11982-3. hdl: 1765/72164 . PMID 8584231. S2CID 7439684.
Bonaldo MF, Lennon G, Soares MB (1997). "Normalization and subtraction: two approaches to facilitate gene discovery". Genome Res. 6 (9): 791–806. doi: 10.1101/gr.6.9.791 . PMID 8889548.
Matuda S, Kodama J, Goshi N, et al. (1998). "A polypeptide derived from mitochondrial dihydrolipoamide succinyltransferase is located on the plasma membrane in skeletal muscle". Biochem. Biophys. Res. Commun. 241 (1): 151–6. doi:10.1006/bbrc.1997.7784. PMID 9405249.
McCartney RG, Rice JE, Sanderson SJ, et al. (1998). "Subunit interactions in the mammalian alpha-ketoglutarate dehydrogenase complex. Evidence for direct association of the alpha-ketoglutarate dehydrogenase and dihydrolipoamide dehydrogenase components". J. Biol. Chem. 273 (37): 24158–64. doi: 10.1074/jbc.273.37.24158 . PMID 9727038.
Sheu KF, Brown AM, Haroutunian V, et al. (1999). "Modulation by DLST of the genetic risk of Alzheimer's disease in a very elderly population". Ann. Neurol. 45 (1): 48–53. doi:10.1002/1531-8249(199901)45:1<48::AID-ART9>3.0.CO;2-V. PMID 9894876. S2CID 40038247.
Ma Q, Chan P, Yang J (2002). "[Association between DLST gene polymorphism and Alzheimer's disease]". Zhonghua Yi Xue Za Zhi. 81 (20): 1246–8. PMID 11825528.
Strausberg RL, Feingold EA, Grouse LH, et al. (2003). "Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences". Proc. Natl. Acad. Sci. U.S.A. 99 (26): 16899–903. Bibcode:2002PNAS...9916899M. doi: 10.1073/pnas.242603899 . PMC 139241 . PMID 12477932.
Kanamori T, Nishimaki K, Asoh S, et al. (2003). "Truncated product of the bifunctional DLST gene involved in biogenesis of the respiratory chain". EMBO J. 22 (12): 2913–23. doi:10.1093/emboj/cdg299. PMC 162151 . PMID 12805207.
Brown AM, Gordon D, Lee H, et al. (2004). "Substantial linkage disequilibrium across the dihydrolipoyl succinyltransferase gene region without Alzheimer's disease association". Neurochem. Res. 29 (3): 629–35. doi:10.1023/B:NERE.0000014833.54481.1d. PMID 15038610. S2CID 35438569.
Gerhard DS, Wagner L, Feingold EA, et al. (2004). "The Status, Quality, and Expansion of the NIH Full-Length cDNA Project: The Mammalian Gene Collection (MGC)". Genome Res. 14 (10B): 2121–7. doi:10.1101/gr.2596504. PMC 528928 . PMID 15489334.
Stelzl U, Worm U, Lalowski M, et al. (2005). "A human protein-protein interaction network: a resource for annotating the proteome". Cell. 122 (6): 957–68. doi:10.1016/j.cell.2005.08.029. hdl: 11858/00-001M-0000-0010-8592-0 . PMID 16169070. S2CID 8235923.
Rual JF, Venkatesan K, Hao T, et al. (2005). "Towards a proteome-scale map of the human protein-protein interaction network". Nature. 437 (7062): 1173–8. Bibcode:2005Natur.437.1173R. doi:10.1038/nature04209. PMID 16189514. S2CID 4427026.

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[WikiPathways-7] The interactive pathway map can be edited at WikiPathways: "TCACycle_WP78".

[refGRCh38Ensembl-1] 1 2 3 GRCh38: Ensembl release 89: ENSG00000119689 - Ensembl, May 2017

[refGRCm38Ensembl-2] 1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000004789 - Ensembl, May 2017

[3] "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[4] "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[pmid8009371-5] Ali G, Wasco W, Cai X, Szabo P, Sheu KF, Cooper AJ, Gaston SM, Gusella JF, Tanzi RE, Blass JP (Jul 1994). "Isolation, characterization, and mapping of gene encoding dihydrolipoyl succinyltransferase (E2k) of human alpha-ketoglutarate dehydrogenase complex". Somat Cell Mol Genet. 20 (2): 99–105. doi:10.1007/BF02290679. PMID 8009371. S2CID 43092605.

[entrez-6] "Entrez Gene: DLST dihydrolipoamide S-succinyltransferase (E2 component of 2-oxo-glutarate complex)".

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[§ 1]

v t e Enzymes: multienzyme complexes
Photosynthesis	Photosynthetic reaction center complex proteins Photosystem I II
Dehydrogenase	Pyruvate dehydrogenase complex E1 E2 E3 Oxoglutarate dehydrogenase OGDH DLST DLD Branched-chain alpha-keto acid dehydrogenase complex BCKDHA BCKDHB DBT DLD
Other	CAD Carbamoyl phosphate synthase II Aspartate carbamoyltransferase Dihydroorotase P450-containing systems Cytochrome b6f complex Electron transport chain Fatty acid synthetase complex Glycine decarboxylase complex Mitochondrial trifunctional protein HADHA HADHB Phosphoenolpyruvate sugar phosphotransferase system Polyketide synthase Sucrase-isomaltase complex Tryptophan synthase

DLST

Contents

Interactive pathway map

Related Research Articles

References

Further reading