1.2.1.105: 2-oxoglutarate dehydrogenase system
This is an abbreviated version!
For detailed information about 2-oxoglutarate dehydrogenase system, go to the full flat file.
Word Map on EC 1.2.1.105
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1.2.1.105
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kgdhc
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dihydrolipoamide
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thiamin
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alpha-ketoacids
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2-oxoadipate
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transsuccinylase
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charcot-marie-tooth
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2-aminoadipic
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medicine
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thiokinase
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glutaryl-coa
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analysis
- 1.2.1.105
- kgdhc
- dihydrolipoamide
- thiamin
- alpha-ketoacids
- 2-oxoadipate
-
transsuccinylase
- charcot-marie-tooth
-
2-aminoadipic
- medicine
- thiokinase
- glutaryl-coa
- analysis
Reaction
Synonyms
2-OGDH2, 2-oxoglutarate dehydrogenase, 2-oxoglutarate dehydrogenase complex, alpha-KDE2, alpha-ketoglutarate dehydrogenase, alpha-ketoglutarate dehydrogenase complex, alpha-KGDH, At3g55410, At5g65750, DHTKD1, dihydrolipoyl succinyltransferase E2, E1a, E1k, E1o, E2, KGDH, KGDHC, More, MPA24.10, ODGH, ODGH1, ODGH2, ODH, OGDC, OGDH, OGDHC, OGDHL, OGHDC-E2, PDHC, SucA, SucB
ECTree
1.2.1.105 2-oxoglutarate dehydrogenase systemAdvanced search results
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results (Engineering
Engineering on EC 1.2.1.105 - 2-oxoglutarate dehydrogenase system
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PROTEIN VARIANTS 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
D114A
additional information
additional information
engineering of the E1 component to accept substrates lacking the 5-carboxylate group. Residue H260 is required for substrate recognition, but H298 can be replaced by hydrophobic residues of similar molecular volume
additional information
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siah2 is the RING finger ubiquitin-protein isopeptide ligase 2, 2-oxoglutarate dehydrogenase component E2 expression and activity are elevated in siah2-/- cells deficient in siah2 expression compared to siah2+/+ cells
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patients bearing the APP670/671 mutation show declined KGDHC activity
additional information
component E1 has two potential Ca2+ -binding sites, site 1 (D114ADLD) and site 2 (E139SDLD). A variant in site 1 (sequence of site 1 is replaced by A114AALA) exhibits an almost complete loss of Ca2+ activation. A variant in site 2 (sequence is replaced with A139SALA) shows no measurable change in Ca2+ sensitivity
additional information
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heterozygous knockout mice deficient in the E3 subunit produce significantly less H2O2 than mitochondria form their littermate wild-type mice
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thiamine diphosphate deficiency diminishes KGDHC activity, knockouts of eNOS or ICAM-1 diminish oxidative stress and reduce neuronal death in thiamine-deficient mice, knockouts of E3 have diminished KGDHC in brain and liver
additional information
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component E1 has two potential Ca2+ -binding sites, site 1 (D114ADLD) and site 2 (E139SDLD). A variant in site 1 (sequence of site 1 is replaced by A114AALA) exhibits an almost complete loss of Ca2+ activation. A variant in site 2 (sequence is replaced with A139SALA) shows no measurable change in Ca2+ sensitivity
