Dihydrolipoyl transsuccinylase
| Dihydrolipoyl transsuccinylase | ||
|---|---|---|
| Properties of human protein | ||
| Mass / length primary structure | 453 amino acids | |
| Cofactor | 2 lipoate | |
| Identifier | ||
| Gene name | DLST | |
| External IDs | ||
| Enzyme classification | ||
| EC, category | 2.3.1.61 , transferase | |
| Response type | Transfer of a succinyl residue | |
| Substrate | CoA + Enzyme-N (6) - (S-Succinyldihydrolipoyl) lysine | |
| Products | Succinyl-CoA + Enzyme-N (6) - (Dihydrolipoyl) lysine | |
| Occurrence | ||
| Parent taxon | Eukaryotes | |
The dihydrolipoyl transsuccinylase (KDH-E2) (exactly: dihydrolipoyllysine residue succinyl transferase ) is an enzyme and one of the three components of the ketoglutarate dehydrogenase - enzyme complex , which is indispensable in the citric acid cycle and in the lysine breakdown of eukaryotes and some bacteria . KDH-E2 catalyzes the transfer of the succinyl residue from the dihydrolipoyllysine amino acid residue belonging to the enzyme to a coenzyme A molecule. It thus functions analogously to dihydrolipoyl transacetylase in the pyruvate dehydrogenase complex (PDH-E2), but has no binding domains for the E1 and E3 units. KDH-E2 forms 24-mers with octahedral symmetry.
A lower production of KDH-E2 in old age or due to mutation may be the cause of neurodegenerative diseases .
literature
- S. Matuda, T. Arimura, et al. a .: A novel protein found in the I bands of myofibrils is produced by alternative splicing of the DLST gene. In: Biochimica et biophysica acta Volume 1800, Number 1, January 2010, pp. 31-39. doi : 10.1016 / j.bbagen.2009.10.003 . PMID 19819302 .
- GE Gibson, JP Blass et al. a .: The alpha-ketoglutarate-dehydrogenase complex: a mediator between mitochondria and oxidative stress in neurodegeneration. In: Molecular neurobiology Volume 31, Numbers 1-3, 2005, pp. 43-63. doi : 10.1385 / MN: 31: 1-3: 043 . PMID 15953811 . (Review).
Individual evidence
- ↑ UniProt P36957
- ↑ Dihydrolipoyl transsuccinylase. In: Online Mendelian Inheritance in Man . (English)
- ↑ L. Yang, Q. Shi et al. a .: Mice deficient in dihydrolipoyl succinyl transferase show increased vulnerability to mitochondrial toxins. In: Neurobiology of Disease Volume 36, Number 2, November 2009, pp. 320-330. doi : 10.1016 / j.nbd.2009.07.023 . PMID 19660549 .
- ↑ M. Dumont, DJ Ho et al. a .: Mitochondrial dihydrolipoyl succinyltransferase deficiency accelerates amyloid pathology and memory deficit in a transgenic mouse model of amyloid deposition. In: Free Radical Biology and Medicine Volume 47, Number 7, October 2009, pp. 1019-1027. doi : 10.1016 / j.freeradbiomed.2009.07.008 . PMID 19596066 . PMC 276114 (free full text).
