| dbo:abstract |
Pantothenatkinasen sind Enzyme, die Pantothensäure phosphorylieren. Dies ist der erste Reaktionsschritt bei der Biosynthese von Coenzym A in allen Lebewesen. Im Menschen sind vier Paraloge des Enzyms bekannt, die jeweils mehrere Isoformen aufweisen (siehe Tabelle). Mutationen im PANK2-Gen sind verantwortlich für die sehr seltene neurodegenerative Erkrankung Pantothenatkinase-assoziierte Neurodegeneration (PKAN), einer Variante von Neurodegeneration mit Eisenablagerung im Gehirn (NBIA). (de) Les pantothénates kinases (PanK; CoaA) sont des enzymes catalysant la phosphorylation du pantothénate (base conjuguée de l'acide pantothénique ou vitamine B5). Il s'agit de la première étape du processus de biosynthèse du coenzyme A. Sont connus à ce jour quatre paralogues de l'enzyme, chacun pouvant avoir plusieurs isoformes (voir ci-dessous). Des mutations du gène PANK2 sont responsables de cas assez rares de neurodégénerescence associée à la pantothénate kinase. (fr) Pantothenate kinase (EC 2.7.1.33, PanK; CoaA) is the first enzyme in the Coenzyme A (CoA) biosynthetic pathway. It phosphorylates pantothenate (vitamin B5) to form 4'-phosphopantothenate at the expense of a molecule of adenosine triphosphate (ATP). It is the rate-limiting step in the biosynthesis of CoA. CoA is a necessary cofactor in all living organisms. It acts as the major acyl group carrier in many important cellular processes, such as the citric acid cycle (tricarboxylic acid cycle) and fatty acid metabolism. Consequently, pantothenate kinase is a key regulatory enzyme in the CoA biosynthetic pathway. (en) パントテン酸キナーゼ（Pantothenate kinase、EC 2.7.1.33）は、補酵素Aの合成に関係する酵素の一つである。パントテン酸のリン酸化反応を触媒して、が合成される。 パントテン酸キナーゼ合成に関する酵素はPANKと呼ばれ、4つ確認されている。このうち、PANK2の変異により、進行性の神経変性疾患である（旧:ハラーフォルデン・スパッツ症候群）が起きる。 (ja) |
| dbo:ecNumber |
2.7.1.33 |
| dbo:thumbnail |
wiki-commons:Special:FilePath/Mechanism_os_pantothenate_kinase.png?width=300 |
| dbo:wikiPageID |
10982938 (xsd:integer) |
| dbo:wikiPageLength |
15534 (xsd:nonNegativeInteger) |
| dbo:wikiPageRevisionID |
1032237015 (xsd:integer) |
| dbo:wikiPageWikiLink |
dbr:Nucleotide dbr:Hydrogen_bond dbr:Valine dbr:Van_der_Waals_force dbc:EC_2.7.1 dbr:Competitive_inhibition dbr:Pseudomonas_aeruginosa dbr:Citric_acid_cycle dbr:Coenzyme_A dbr:Enzyme dbr:Glutamic_acid dbr:Mitochondrion dbr:Concerted_reaction dbr:Cooperativity dbr:Lysine dbr:Malonyl-CoA dbr:Staphylococcus dbr:Staphylococcus_aureus dbr:Fatty_acid dbr:Transition_state dbr:Acyl-CoA dbr:Acyl_group dbr:Tyrosine dbr:Acetyl-CoA dbr:Adenine dbr:Adenosine_diphosphate dbr:Adenosine_triphosphate dbr:Pantothenic_acid dbr:Protein_domain dbr:Histidine dbr:Acyl dbr:Arginine dbr:Asparagine dbr:Aspartic_acid dbr:Cofactor_(biochemistry) dbr:C-terminus dbr:Phenylalanine dbr:Phosphorane dbr:Metabolism dbr:Methionine dbr:Serine dbr:Hydroxyl dbr:Exon dbr:N-terminus dbr:PANK4 dbr:PANK2 dbr:Pantothenate_kinase-associated_neurodegeneration dbr:Thiol dbr:Walker_motifs dbr:PANK1 dbr:Pantothenate dbr:P-loop dbr:File:Human_PanK-II_structure.png dbr:File:Mechanism_os_pantothenate_kinase.png dbr:File:PanK-III.png dbr:File:Pantothenate_Kinase_mechanism.png |
| dbp:casNumber |
9026 (xsd:integer) |
| dbp:ecNumber |
2.700000 (xsd:double) |
| dbp:iubmbEcNumber |
2 (xsd:integer) |
| dbp:name |
Pantothenate kinase (en) |
| dbp:wikiPageUsesTemplate |
dbt:EC_number dbt:Enzymes dbt:Gene dbt:Infobox_enzyme dbt:Kinases dbt:MeshName dbt:Portal_bar dbt:Reflist dbt:Metabolism_of_vitamins,_coenzymes,_and_cofactors |
| dcterms:subject |
dbc:EC_2.7.1 |
| gold:hypernym |
dbr:Enzyme |
| rdf:type |
owl:Thing dbo:Biomolecule wikidata:Q206229 wikidata:Q8047 yago:Abstraction100002137 yago:Activator114723079 yago:Catalyst114723628 yago:Chemical114806838 yago:Compound114818238 yago:Enzyme114732946 yago:Macromolecule114944888 yago:Material114580897 yago:Matter100020827 yago:Molecule114682133 yago:OrganicCompound114727670 yago:Part113809207 yago:PhysicalEntity100001930 yago:Protein114728724 yago:Relation100031921 dbo:Enzyme dbo:Protein yago:Substance100019613 yago:Thing100002452 yago:Unit109465459 |
| rdfs:comment |
Pantothenatkinasen sind Enzyme, die Pantothensäure phosphorylieren. Dies ist der erste Reaktionsschritt bei der Biosynthese von Coenzym A in allen Lebewesen. Im Menschen sind vier Paraloge des Enzyms bekannt, die jeweils mehrere Isoformen aufweisen (siehe Tabelle). Mutationen im PANK2-Gen sind verantwortlich für die sehr seltene neurodegenerative Erkrankung Pantothenatkinase-assoziierte Neurodegeneration (PKAN), einer Variante von Neurodegeneration mit Eisenablagerung im Gehirn (NBIA). (de) Les pantothénates kinases (PanK; CoaA) sont des enzymes catalysant la phosphorylation du pantothénate (base conjuguée de l'acide pantothénique ou vitamine B5). Il s'agit de la première étape du processus de biosynthèse du coenzyme A. Sont connus à ce jour quatre paralogues de l'enzyme, chacun pouvant avoir plusieurs isoformes (voir ci-dessous). Des mutations du gène PANK2 sont responsables de cas assez rares de neurodégénerescence associée à la pantothénate kinase. (fr) パントテン酸キナーゼ（Pantothenate kinase、EC 2.7.1.33）は、補酵素Aの合成に関係する酵素の一つである。パントテン酸のリン酸化反応を触媒して、が合成される。 パントテン酸キナーゼ合成に関する酵素はPANKと呼ばれ、4つ確認されている。このうち、PANK2の変異により、進行性の神経変性疾患である（旧:ハラーフォルデン・スパッツ症候群）が起きる。 (ja) Pantothenate kinase (EC 2.7.1.33, PanK; CoaA) is the first enzyme in the Coenzyme A (CoA) biosynthetic pathway. It phosphorylates pantothenate (vitamin B5) to form 4'-phosphopantothenate at the expense of a molecule of adenosine triphosphate (ATP). It is the rate-limiting step in the biosynthesis of CoA. (en) |
| rdfs:label |
Pantothenatkinase (de) Pantothénate kinase (fr) パントテン酸キナーゼ (ja) Pantothenate kinase (en) |
| owl:sameAs |
freebase:Pantothenate kinase wikidata:Pantothenate kinase dbpedia-de:Pantothenate kinase dbpedia-fr:Pantothenate kinase dbpedia-ja:Pantothenate kinase dbpedia-kk:Pantothenate kinase dbpedia-sh:Pantothenate kinase dbpedia-sr:Pantothenate kinase https://global.dbpedia.org/id/3opFw |
| prov:wasDerivedFrom |
wikipedia-en:Pantothenate_kinase?oldid=1032237015&ns=0 |
| foaf:depiction |
wiki-commons:Special:FilePath/Human_PanK-II_structure.png wiki-commons:Special:FilePath/Mechanism_os_pantothenate_kinase.png wiki-commons:Special:FilePath/PanK-III.png wiki-commons:Special:FilePath/Pantothenate_Kinase_mechanism.png |
| foaf:isPrimaryTopicOf |
wikipedia-en:Pantothenate_kinase |
| foaf:name |
Pantothenate kinase (en) |
| is dbo:wikiPageRedirects of |
dbr:EC_2.7.1.33 dbr:ATP:(R)-pantothenate_4'-phosphotransferase |
| is dbo:wikiPageWikiLink of |
dbr:Coenzyme_A dbr:Pantothenic_acid dbr:List_of_EC_numbers_(EC_2) dbr:Cofactor_engineering dbr:PANK2_(gene) dbr:PANK4 dbr:Pantothenate_kinase-associated_neurodegeneration dbr:PANK1 dbr:EC_2.7.1.33 dbr:ATP:(R)-pantothenate_4'-phosphotransferase |
| is foaf:primaryTopic of |
wikipedia-en:Pantothenate_kinase |
