Lysyl hydroxylase (original) (raw)

dbo:abstract

La lisil hidroxilasa o procolágeno-lisina 4-dioxigenasa, es una enzima que cataliza la hidroxilación de la lisina a hidroxilisina. (es)
Die Lysylhydroxylasen sind Enzyme, die die Hydroxylierung von Lysinresten in Proteinen katalysieren. Die Lysylhydroxylasen erzeugen aus Lysinresten Hydroxylysin als posttranslationale Modifikation, unter anderem bei der Stabilisierung von Kollagen. Cofaktoren sind Eisenionen und Ascorbinsäure (Vitamin C). Lysylhydroxylasen sind Dimere und im Lumen des rauen endoplasmatischen Retikulums lokalisiert. Ein Mangel an Vitamin C führt zu Defekten in der Funktion des Kollagens, die zu den Symptomen des Skorbuts gerechnet werden. (de)
Lysyl hydroxylases (or procollagen-lysine 5-dioxygenases) are alpha-ketoglutarate-dependent hydroxylases enzymes that catalyze the hydroxylation of lysine to hydroxylysine. Lysyl hydroxylases require iron and vitamin C as cofactors for their oxidation activity. It takes place (as a post-translational modification) following collagen synthesis in the cisternae (lumen) of the rough endoplasmic reticulum (ER). There are three lysyl hydroxylases (LH1-3) encoded in the human genome, namely: PLOD1, PLOD2 and PLOD3. From PLOD2 two splice variant can be expressed (LH2a and LH2b), where LH2b differs from LH2a by incorporating the small exon 13A. LH1 and LH3 hydroxylate lysyl residues in the collagen triple helix, whereas LH2b hydroxylates lysyl residues in the telopeptides of collagen. In addition to its hydroxylation activity, LH3 has glycosylation activity that produces either monosaccharide (Gal) or disaccharide (Glc-Gal) attached to collagen hydroxylysines. Collagen lysyl hydroxylation is the first step in collagen pyridinoline cross-linking, that is necessary for the stabilization of collagen. (en)
La lysine hydroxylase est une oxydoréductase qui catalyse la réaction : L-lysine-[procollagène] + α-cétoglutarate + O2 (2S,5R)- 5-hydroxy-L-lysine-[procollagène] + succinate + CO2. Cette enzyme homodimérique est liée à la membrane du réticulum endoplasmique rugueux du côté du lumen ; elle est indispensable à la formation et à la stabilisation du collagène. Elle requiert du fer et du L-ascorbate (vitamine C) comme cofacteurs. (fr)
リシルヒドロキシラーゼ（lysyl hydroxylase）は、リシンをヒドロキシル化し、ヒドロキシリシンにする酸化還元酵素である。この酵素反応はコラーゲンの形成・安定に必須であり、翻訳後修飾として、タンパク質の合成のあとで起こる。この酵素は膜結合性二量体酵素で、粗面小胞体のルーメン（内腔）に局在している。補因子として鉄とビタミンCを必要とする。 (ja)
La procollagene-lisina 5-diossigenasi (nota comunemente anche con il nome di lisil idrossilasi) è un enzima appartenente alla classe delle ossidoreduttasi, che catalizza la seguente reazione: L-lisina + 2-ossoglutarato + O2 ⇄ procollagene 5-idrossi-L-lisina + succinato + CO2 L'enzima catalizza la reazione di idrossilazione (l'aggiunta di un ) di un di lisina formando l'amminoacido modificato . Questo amminoacido modificato si trova nelle molecole di collagene dove partecipa alla formazione di legami crociati tra le catene alfa del collagene e tra le molecole di nelle fibe di collagene stabilizzando queste molecole. Per il funzionamento di questo enzima è necessario un atomo di ferro allo stato di ossidazione +2 (Fe2+), ossigeno molecolare e ascorbato (vitamina C). (it)
Lysylhydroxylases zijn enzymen die lysine hydroxyleren. De meest bekende lysylhydroxylases zijn de lysylhydroxylases (LH). Er zijn drie varianten bekend (LH1, LH2 en LH3) die door drie verschillende genen gecodeerd zijn, namelijk PLOD1, PLOD2 en PLOD3. Mutaties in het PLOD2-gen worden wel in verband gebracht met het syndroom van Bruck. Een tekort aan de cofactor van lysylhydroxylases, vitamine C leidt tot scheurbuik. (nl)

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LLH, PLOD (en)

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p (en)
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procollagen-lysine, 2-oxoglutarate 5-dioxygenase 2 (en)
procollagen-lysine 1, 2-oxoglutarate 5-dioxygenase 1 (en)

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153454 (xsd:integer)
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dbp:refseq

NM_000302 (en)
NM_000935 (en)

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PLOD1 (en)
PLOD2 (en)

dbp:uniprot

O00469 (en)
Q02809 (en)

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dcterms:subject

gold:hypernym

dbr:Enzyme

rdf:type

rdfs:comment

La lisil hidroxilasa o procolágeno-lisina 4-dioxigenasa, es una enzima que cataliza la hidroxilación de la lisina a hidroxilisina. (es)
Die Lysylhydroxylasen sind Enzyme, die die Hydroxylierung von Lysinresten in Proteinen katalysieren. Die Lysylhydroxylasen erzeugen aus Lysinresten Hydroxylysin als posttranslationale Modifikation, unter anderem bei der Stabilisierung von Kollagen. Cofaktoren sind Eisenionen und Ascorbinsäure (Vitamin C). Lysylhydroxylasen sind Dimere und im Lumen des rauen endoplasmatischen Retikulums lokalisiert. Ein Mangel an Vitamin C führt zu Defekten in der Funktion des Kollagens, die zu den Symptomen des Skorbuts gerechnet werden. (de)
La lysine hydroxylase est une oxydoréductase qui catalyse la réaction : L-lysine-[procollagène] + α-cétoglutarate + O2 (2S,5R)- 5-hydroxy-L-lysine-[procollagène] + succinate + CO2. Cette enzyme homodimérique est liée à la membrane du réticulum endoplasmique rugueux du côté du lumen ; elle est indispensable à la formation et à la stabilisation du collagène. Elle requiert du fer et du L-ascorbate (vitamine C) comme cofacteurs. (fr)
リシルヒドロキシラーゼ（lysyl hydroxylase）は、リシンをヒドロキシル化し、ヒドロキシリシンにする酸化還元酵素である。この酵素反応はコラーゲンの形成・安定に必須であり、翻訳後修飾として、タンパク質の合成のあとで起こる。この酵素は膜結合性二量体酵素で、粗面小胞体のルーメン（内腔）に局在している。補因子として鉄とビタミンCを必要とする。 (ja)
Lysylhydroxylases zijn enzymen die lysine hydroxyleren. De meest bekende lysylhydroxylases zijn de lysylhydroxylases (LH). Er zijn drie varianten bekend (LH1, LH2 en LH3) die door drie verschillende genen gecodeerd zijn, namelijk PLOD1, PLOD2 en PLOD3. Mutaties in het PLOD2-gen worden wel in verband gebracht met het syndroom van Bruck. Een tekort aan de cofactor van lysylhydroxylases, vitamine C leidt tot scheurbuik. (nl)
Lysyl hydroxylases (or procollagen-lysine 5-dioxygenases) are alpha-ketoglutarate-dependent hydroxylases enzymes that catalyze the hydroxylation of lysine to hydroxylysine. Lysyl hydroxylases require iron and vitamin C as cofactors for their oxidation activity. It takes place (as a post-translational modification) following collagen synthesis in the cisternae (lumen) of the rough endoplasmic reticulum (ER). There are three lysyl hydroxylases (LH1-3) encoded in the human genome, namely: PLOD1, PLOD2 and PLOD3. From PLOD2 two splice variant can be expressed (LH2a and LH2b), where LH2b differs from LH2a by incorporating the small exon 13A. LH1 and LH3 hydroxylate lysyl residues in the collagen triple helix, whereas LH2b hydroxylates lysyl residues in the telopeptides of collagen. In addition (en)
La procollagene-lisina 5-diossigenasi (nota comunemente anche con il nome di lisil idrossilasi) è un enzima appartenente alla classe delle ossidoreduttasi, che catalizza la seguente reazione: L-lisina + 2-ossoglutarato + O2 ⇄ procollagene 5-idrossi-L-lisina + succinato + CO2 L'enzima catalizza la reazione di idrossilazione (l'aggiunta di un ) di un di lisina formando l'amminoacido modificato . Per il funzionamento di questo enzima è necessario un atomo di ferro allo stato di ossidazione +2 (Fe2+), ossigeno molecolare e ascorbato (vitamina C). (it)

rdfs:label

Lysylhydroxylasen (de)
Lisil hidroxilasa (es)
Lysine hydroxylase (fr)
Procollagene-lisina 5-diossigenasi (it)
リシルヒドロキシラーゼ (ja)
Lysyl hydroxylase (en)
Lysylhydroxylase (nl)

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wikipedia-en:Lysyl_hydroxylase?oldid=1116912210&ns=0

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