Literature summary for 2.7.1.94 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 .

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Protein Variants

Protein Variants	Comment	Organism
G126E	inactive	Homo sapiens

Localization

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

Natural Substrates/ Products (Substrates)

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

Organism

Organism	UniProt	Comment	Textmining
Homo sapiens	Q53H12	-	-

Substrates and Products (Substrate)

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

Synonyms

Synonyms	Comment	Organism
AGK	-	Homo sapiens

General Information

General Information	Comment	Organism
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)