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Literature summary for 1.14.11.4 extracted from

  • Hyry, M.; Lantto, J.; Myllyharju, J.
    Missense mutations that cause Bruck syndrome affect enzymatic activity, folding, and oligomerization of lysyl hydroxylase 2 (2009), J. Biol. Chem., 284, 30917-30924.
    View publication on PubMedView publication on EuropePMC
Search for more literature for 1.14.11.4

Activating Compound

Activating Compound Comment Organism Structure
ascorbic acid activates Homo sapiens

Cloned(Commentary)

Cloned (Comment) Organism
expression of wild-type and mutantenzymes in Spodoptera frugiperda Sf9 cells using the baculovirus transfection method Homo sapiens

Protein Variants

Protein Variants Comment Organism
G597V naturally occuring recessive point mutation, leads to abnormal folding and oligomerization of the mutant enzyme, which shows over 95% reduced activity compared to the wild-type enzyme Homo sapiens
R594H naturally occuring recessive point mutation, leads to abnormal folding and oligomerization of the mutant enzyme, which shows over 95% reduced activity compared to the wild-type enzyme Homo sapiens
T604I naturally occuring recessive point mutation, leads 70-92% reduced activity, dependent on the 2-oxoglutarate concentration, compared top the wild-type due to a 10fold increase in the Km for 2-oxoglutarate, the mutant shows unaltered folding and oligomerization. The Km values of the T604I mutant for the peptide substrate, Fe2+, and ascorbate are identical to those of the wild-type Homo sapiens

KM Value [mM]

KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
0.025
-
 2-oxoglutarate wild-type enzyme Homo sapiens
0.25
-
 2-oxoglutarate mutant T604I Homo sapiens
0.4
-
 (IKG)3 wild-type enzyme and mutant T604I Homo sapiens
5
-
 (IKG)3 above, mutant G597V Homo sapiens
5
-
 (IKG)3 above, mutant R594H Homo sapiens

Metals/Ions

Metals/Ions Comment Organism Structure
Fe2+ activates Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
additional information Homo sapiens the enzyme is responsible for the hydroxylation of collagen telopeptides ?
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
 isozyme LH2
-

Purification (Commentary)

Purification (Comment) Organism
recombinant wild-type and mutant enzymes from Sf9 insect cells by chelating affinity chromatography Homo sapiens

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
(IKG)3 + 2-oxoglutarate + O2
-
 Homo sapiens ?
-
 ?
additional information the enzyme is responsible for the hydroxylation of collagen telopeptides Homo sapiens ?
-
 ?

Synonyms

Synonyms Comment Organism
lysyl hydroxylase 2
-
 Homo sapiens

General Information

General Information Comment Organism
physiological function the enzyme is responsible for the hydroxylation of collagen telopeptides Homo sapiens