Literature summary for 2.4.1.66 extracted from

Ruotsalainen, H.; Sipilae, L.; Vapola, M.; Sormunen, R.; Salo, A.M.; Uitto, L.; Mercer, D.K.; Robins, S.P.; Risteli, M.; Aszodi, A.; Faessler, R.; Myllylae, R.
Glycosylation catalyzed by lysyl hydroxylase 3 is essential for basement membranes (2006), J. Cell Sci., 119, 625-635.

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Natural Substrates/ Products (Substrates)

Natural Substrates	Organism	Comment (Nat. Sub.)	Natural Products	Comment (Nat. Pro.)	Rev.	Reac.
additional information	Mus musculus	the reduction of the galactosylhydroxylysyl glucosyltransferase activity of LH3 disrupts the localization of type IV collagen, and thus the formation of basement membranes during mouse embryogenesis leading to lethality at embryonic day 9.5-14.5. Survival of hypomorphic embryos and the formation of the basement membrane are directly correlated with the level of galactosylhydroxylysyl glucosyltransferase activity. An LH3-knockout mouse lacks galactosylhydroxylysyl glucosyltransferase. Lysyl hydroxylase 3 is the main molecule responsible for galactosylhydroxylysyl glucosyltransferase activity. Galactosylhydroxylysyl glucosyltransferase is essential for the formation of the basement membrane	?	-	?

Organism

Organism	UniProt	Comment	Textmining
Mus musculus	-	-	-

Source Tissue

Source Tissue	Comment	Organism	Textmining
additional information	the reduction of the galactosylhydroxylysyl glucosyltransferase activity of LH3 disrupts the localization of type IV collagen, and thus the formation of basement membranes during mouse embryogenesis leading to lethality at embryonic day 9.5-14.5. Survival of hypomorphic embryos and the formation of the basement membrane are directly correlated with the level of galactosylhydroxylysyl glucosyltransferase activity. An LH3-knockout mouse lacks galactosylhydroxylysyl glucosyltransferase. Lysyl hydroxylase 3 is the main molecule responsible for galactosylhydroxylysyl glucosyltransferase activity. Galactosylhydroxylysyl glucosyltransferase is essential for the formation of the basement membrane	Mus musculus	-

Substrates and Products (Substrate)

Substrates	Comment Substrates	Organism	Products	Comment (Products)	Rev.	Reac.
additional information	the reduction of the galactosylhydroxylysyl glucosyltransferase activity of LH3 disrupts the localization of type IV collagen, and thus the formation of basement membranes during mouse embryogenesis leading to lethality at embryonic day 9.5-14.5. Survival of hypomorphic embryos and the formation of the basement membrane are directly correlated with the level of galactosylhydroxylysyl glucosyltransferase activity. An LH3-knockout mouse lacks galactosylhydroxylysyl glucosyltransferase. Lysyl hydroxylase 3 is the main molecule responsible for galactosylhydroxylysyl glucosyltransferase activity. Galactosylhydroxylysyl glucosyltransferase is essential for the formation of the basement membrane	Mus musculus	?	-	?

Synonyms

Synonyms	Comment	Organism
galactosylhydroxylysyl glucosyltransferase	-	Mus musculus
lysyl hydroxylase 3	(LH3) is a multifunctional enzyme possessing lysyl hydroxylase (LH), hydroxylysyl galactosyltransferase (GT) and galactosylhydroxylysyl glucosyltransferase (GGT) activities in vitro	Mus musculus

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Release 2023.1 (January 2023)