Literature summary extracted from

Kang, Y.; Stroud, D.A.; Baker, M.J.; De Souza, D.P.; Frazier, A.E.; Liem, M.; Tull, D.; Mathivanan, S.; McConville, M.J.; Thorburn, D.R.; Ryan, M.T.; Stojanovski, D.
Sengers syndrome-associated mitochondrial acylglycerol kinase is a subunit of the human TIM22 protein import complex (2017), Mol. Cell, 67, 457-470.e5 .

Protein Variants

EC Number	Protein Variants	Comment	Organism
2.7.1.94	G126E	inactive	Homo sapiens

Localization

EC Number	Localization	Comment	Organism	GeneOntology No.	Textmining
2.7.1.94	mitochondrion	-	Homo sapiens	5739	-

Natural Substrates/ Products (Substrates)

EC Number	Natural Substrates	Organism	Comment (Nat. Sub.)	Natural Products	Comment (Nat. Pro.)	Rev.	Reac.
2.7.1.94	ATP + acylglycerol	Homo sapiens	-	ADP + lysophosphatidic acid	-	?

Organism

EC Number	Organism	UniProt	Comment	Textmining
2.7.1.94	Homo sapiens	Q53H12	-	-

Substrates and Products (Substrate)

EC Number	Substrates	Comment Substrates	Organism	Products	Comment (Products)	Rev.	Reac.
2.7.1.94	ATP + acylglycerol	-	Homo sapiens	ADP + lysophosphatidic acid	-	?

Synonyms

EC Number	Synonyms	Comment	Organism
2.7.1.94	AGK	-	Homo sapiens

General Information

EC Number	General Information	Comment	Organism
2.7.1.94	physiological function	the kinase activity of the enzyme is dispensable for protein import but required for the structural integrity of mitochondria and apoptotic resistance. The enzyme functions as a subunit of the carrier translocase TIM22 complex independent of its kinase activity. The enzyme mutated in Sengers syndrome is a subunit of the TIM22 complex. The enzyme is required for the import of membrane proteins ANT1 and SLC25A24 into mitochondria	Homo sapiens

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Release 2023.1 (January 2023)