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Hephästin – Wikipedia Zum Inhalt springen

Hephästin

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{{#if: | {{#if: | {{#if:4866| {{#if: 1136 Aminosäuren | {{#if: Monomer; single pass Typ 1 Membranprotein | {{#if: 6 Kupfer | {{#if: | {{#if: 2 | {{#if: HEPHKIAA0698Q9BQS7300167 | {{#if: HEPHKIAA0698 | {{#if: Q9BQS7300167 | {{#if: | {{#if:| {{#if: | {{#if: 1.-.-.-OxidoreduktaseOxidation4 Fe2+ + 4 H+ + O24 Fe3+ + 2 H2O | {{#if: 1.-.-.-Oxidoreduktase| {{#if: | {{#if: | {{#if: Oxidation| {{#if: 4 Fe2+ + 4 H+ + O2| {{#if: 4 Fe3+ + 2 H2O| {{#if: Neumünder{{#if: Q9BQS7 | {{#ifeq: Ferroxidase-like | NV ||1}}|}}| {{#if: Q9BQS7 | {{#ifeq: Ferroxidase-like | NV || | style="background:#C3FDB8; color:#000000;" | Homologie-Familie {{#if: Neumünder | | style="background:#C3FDB8; color:#000000;" | Übergeordnetes Taxon {{#if: | | style="background:#C3FDB8; color:#000000;" | Ausnahmen {{#if: {{#if: Hausmaus | | colspan="3" style="background:#90EE90; color:#202122; text-align:center;" | Orthologe {{#if: ENSG00000089472ENSMUSG00000031209
{{#if: | | Hephästin }}
[[Datei:|250x200px|{{#if: | | Hephästin }}]]
Hephästin }}
Andere Namen

{{{Andere Namen}}} }}

Vorhandene Strukturdaten: }}

Eigenschaften des menschlichen Proteins

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Masse/Länge Primärstruktur 1136 Aminosäuren

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Sekundär- bis Quartärstruktur Monomer; single pass Typ 1 Membranprotein

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Kofaktor 6 Kupfer

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Präkursor

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Isoformen 2

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Bezeichner
{{#if:KIAA0698 | Gen-Namen | Gen-Name}} | Gen-Name(n) }} {{#if:4866 | HEPH | HEPH}}{{#if:KIAA0698 |, KIAA0698}} }}
Externe IDs

{{#if: |

0 | }} - = – valid|1|2=/^[1-9]%d?%d?%d?%d?%d?%d?%d?%d?$/ 0 | Wikipedia:Vorlagenfehler/Vorlage:PubChem}} template= Vorlage:PubChem format=

}} }}{{#invoke:TemplatePar|check

all= 1= opt= 2= 0 | Wikipedia:Vorlagenfehler/Vorlage:PubChem}} template= Vorlage:PubChem format=

}}{{#if: {{#invoke:Wikidata|pageId}}||{{#ifeq: 0 | 0 | }} }}}}{{#if: | 

 }}

}}

Arzneistoffangaben
ATC-Code 
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 {{#if:|
DrugBank [2] 
 }}
 {{#if:|
Wirkstoffklasse 
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}}

Transporter-Klassifikation
TCDB [3] 
 {{#if: |
Bezeichnung 
 }}

}}

{{#if:|Inhibitorklassifikation|{{#if:|Enzymklassifikationen|Enzymklassifikation}}}}
EC, Kategorie {{#if:1.-.-.-| 1.-.-.-}}{{#if: Oxidoreduktase|, Oxidoreduktase}} }}
MEROPS [4]}}
MEROPS {{{Inhibitor_fam}}}}}
Reaktionsart Oxidation 
 }}
Substrat 4 Fe2+ + 4 H+ + O2 
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Produkte 4 Fe3+ + 2 H2O 
 }}


}}

Vorkommen
{{#if: Ferroxidase-like|Hovergen}}] Ferroxidase-like|Hovergen}}]}} }} }}
Neumünder }}
}}

}}

Mensch Hausmaus
Entrez 
 {{ #if: 9843
9843 }} 
 {{ #if: 15203
15203 }}
style="background:#C3FDB8; color:#202122;" | Ensembl 
    {{ #if: ENSG00000089472
ENSG00000089472 }} 
    {{ #if: ENSMUSG00000031209
ENSMUSG00000031209 }}

} 

 |-
 | style="background:#C3FDB8; color:#202122;" | UniProt
 {{ #if: Q9BQS7
    | | {{#if: kurz| | UniProt }} {{#if:|{{{titel}}}|Q9BQS7}}{{#if:|Vorlage:Abrufdatum}}
    | |  }}
 {{ #if: Q9Z0Z4
    | | {{#if: kurz| | UniProt }} {{#if:|{{{titel}}}|Q9Z0Z4}}{{#if:|Vorlage:Abrufdatum}}
    | |  }}
 |-
 {{#if: NM_001130860NM_001159627
    | | style="background:#C3FDB8; color:#202122;" | Refseq (mRNA)
      {{ #if: NM_001130860
         | | NM_001130860
         | |  }}
      {{ #if: NM_001159627
         | | NM_001159627
         | |  }}
    |}}
 |-
 {{#if: NP_001124332NP_001153099
    | | style="background:#C3FDB8; color:#202122;" | Refseq (Protein)
      {{ #if: NP_001124332
         | | NP_001124332
         | |  }}
      {{ #if: NP_001153099
         | | NP_001153099
         | |  }}
    |}}
 |-
 {{#if: X6616251966267389X9645543696574485
    | | style="background:#C3FDB8; color:#202122;" | Genlocus
      {{#if: X 
        | {{#if: 66162519 
            | {{#if: 66267389 
                | |  #if:  |?db=|}}&position=chrX:66162519-66267389 Chr X: {{#expr: 66162519 / 1000000 round 2}} – {{#expr: 66267389 / 1000000 round 2}} Mb 
                | |  }}
            | |  }}
        | |  }}
      {{#if: X 
        | {{#if: 96455436 
            | {{#if: 96574485 
                | |  #if:  |?db=|}}&position=chrX:96455436-96574485 Chr X: {{#expr: 96455436 / 1000000 round 2}} – {{#expr: 96574485 / 1000000 round 2}} Mb 
                | |  }}
           | |  }}
       | |  }}
    |}}
 |-
 | style="background:#C3FDB8; color:#202122;" | PubMed-Suche 
 {{ #if: 9843
    | | 9843
    | |  }}
 {{ #if: 15203
    | | 15203
    | |  }}

| |- | colspan="3" style="background:#90EE90; color:#202122; text-align:center;" | Orthologe (Mensch) |- | style="background:#C3FDB8; color:#202122;" | Entrez | colspan="2" | 9843 

 {{#if: ENSG00000089472
    | |-

| style="background:#C3FDB8; color:#202122;" | Ensembl | colspan="2" | ENSG00000089472 }} |- | style="background:#C3FDB8; color:#202122;" | UniProt | colspan="2" | {{#if: kurz| | UniProt }} {{#if:|{{{titel}}}|Q9BQS7}}{{#if:|Vorlage:Abrufdatum}} 

 {{#if: NM_001130860
    | |-

| style="background:#C3FDB8; color:#202122;" | Refseq (mRNA) | colspan="2" | NM_001130860 }} 

 {{#if: NP_001124332
    | |-

| style="background:#C3FDB8; color:#202122;" | Refseq (Protein) | colspan="2" | NP_001124332 }} 

 {{#if: X 
   | {{#if: 66162519 
       | {{#if: 66267389 
           | |-

| style="background:#C3FDB8; color:#202122;" | Genlocus | colspan="2" | #if: |?db=|}}&position=chrX:66162519-66267389 Chr X: {{#expr: 66162519 / 1000000 round 2}} – {{#expr: 66267389 / 1000000 round 2}} Mb 

           |  }}
       |  }}
   |  }}

|- | style="background:#C3FDB8; color:#202122;" | PubMed-Suche | colspan="2" | 9843 }} {{#if: Hausmaus 

    | {{#if: 15203ENSMUSG00000031209NM_001159627NP_001153099X9645543696574485Q9Z0Z4 
        |
        |  Spezies2= ist definiert, es sind jedoch keine weiteren Angaben zu Spezies2 vorhanden.{{#ifeq:0|0| }} }}
    | {{#if: 15203ENSMUSG00000031209NM_001159627NP_001153099X9645543696574485Q9Z0Z4 
        |  Es sind Angaben zu Spezies2 vorhanden, Spezies2= ist jedoch nicht definiert.{{#ifeq:0|0| }}
        |}}

}} | {{#if: Hausmaus | | colspan="3" style="background:#90EE90; color:#202122; text-align:center;" | Orthologe 

 |-
 | style="background:#C3FDB8; color:#202122;" |
 | style="background:#C3FDB8; color:#202122; text-align:center;" | Mensch
 | style="background:#C3FDB8; color:#202122; text-align:center;" | Hausmaus
 |-
 | style="background:#C3FDB8; color:#202122;" | Entrez
 {{ #if: 9843
    | | 9843
    | |  }}
 {{ #if: 15203
    | | 15203
    | |  }}
 |-
 {{#if: ENSG00000089472ENSMUSG00000031209
    | | style="background:#C3FDB8; color:#202122;" | Ensembl
    {{ #if: ENSG00000089472
       || ENSG00000089472
       ||  }}
    {{ #if: ENSMUSG00000031209
       || ENSMUSG00000031209
       ||  }}
  |}}
 |-
 | style="background:#C3FDB8; color:#202122;" | UniProt
 {{ #if: Q9BQS7
    | | {{#if: kurz| | UniProt }} {{#if:|{{{titel}}}|Q9BQS7}}{{#if:|Vorlage:Abrufdatum}}
    | |  }}
 {{ #if: Q9Z0Z4
    | | {{#if: kurz| | UniProt }} {{#if:|{{{titel}}}|Q9Z0Z4}}{{#if:|Vorlage:Abrufdatum}}
    | |  }}
 |-
 {{#if: NM_001130860NM_001159627
    | | style="background:#C3FDB8; color:#202122;" | Refseq (mRNA)
      {{ #if: NM_001130860
         | | NM_001130860
         | |  }}
      {{ #if: NM_001159627
         | | NM_001159627
         | |  }}
    |}}
 |-
 {{#if: NP_001124332NP_001153099
    | | style="background:#C3FDB8; color:#202122;" | Refseq (Protein)
      {{ #if: NP_001124332
         | | NP_001124332
         | |  }}
      {{ #if: NP_001153099
         | | NP_001153099
         | |  }}
    |}}
 |-
 {{#if: X6616251966267389X9645543696574485
    | | style="background:#C3FDB8; color:#202122;" | Genlocus
      {{#if: X 
        | {{#if: 66162519 
            | {{#if: 66267389 
                | |  #if:  |?db=|}}&position=chrX:66162519-66267389 Chr X: {{#expr: 66162519 / 1000000 round 2}} – {{#expr: 66267389 / 1000000 round 2}} Mb 
                | |  }}
            | |  }}
        | |  }}
      {{#if: X 
        | {{#if: 96455436 
            | {{#if: 96574485 
                | |  #if:  |?db=|}}&position=chrX:96455436-96574485 Chr X: {{#expr: 96455436 / 1000000 round 2}} – {{#expr: 96574485 / 1000000 round 2}} Mb 
                | |  }}
           | |  }}
       | |  }}
    |}}
 |-
 | style="background:#C3FDB8; color:#202122;" | PubMed-Suche 
 {{ #if: 9843
    | | 9843
    | |  }}
 {{ #if: 15203
    | | 15203
    | |  }}

| |- | colspan="3" style="background:#90EE90; color:#202122; text-align:center;" | Orthologe (Mensch) |- | style="background:#C3FDB8; color:#202122;" | Entrez | colspan="2" | 9843 

 {{#if: ENSG00000089472
    | |-

| style="background:#C3FDB8; color:#202122;" | Ensembl | colspan="2" | ENSG00000089472 }} |- | style="background:#C3FDB8; color:#202122;" | UniProt | colspan="2" | {{#if: kurz| | UniProt }} {{#if:|{{{titel}}}|Q9BQS7}}{{#if:|Vorlage:Abrufdatum}} 

 {{#if: NM_001130860
    | |-

| style="background:#C3FDB8; color:#202122;" | Refseq (mRNA) | colspan="2" | NM_001130860 }} 

 {{#if: NP_001124332
    | |-

| style="background:#C3FDB8; color:#202122;" | Refseq (Protein) | colspan="2" | NP_001124332 }} 

 {{#if: X 
   | {{#if: 66162519 
       | {{#if: 66267389 
           | |-

| style="background:#C3FDB8; color:#202122;" | Genlocus | colspan="2" | #if: |?db=|}}&position=chrX:66162519-66267389 Chr X: {{#expr: 66162519 / 1000000 round 2}} – {{#expr: 66267389 / 1000000 round 2}} Mb 

           |  }}
       |  }}
   |  }}

|- | style="background:#C3FDB8; color:#202122;" | PubMed-Suche | colspan="2" | 9843 }} {{#if: Hausmaus 

    | {{#if: 15203ENSMUSG00000031209NM_001159627NP_001153099X9645543696574485Q9Z0Z4 
        |
        |  Spezies2= ist definiert, es sind jedoch keine weiteren Angaben zu Spezies2 vorhanden.{{#ifeq:0|0| }} }}
    | {{#if: 15203ENSMUSG00000031209NM_001159627NP_001153099X9645543696574485Q9Z0Z4 
        |  Es sind Angaben zu Spezies2 vorhanden, Spezies2= ist jedoch nicht definiert.{{#ifeq:0|0| }}
        |}}

}} }}

|}{{#if:||}}

Hephästin (Gen: HEPH) ist ein homologes Enzym des Caeruloplasmin, ein Membranprotein, das in allen Neumündern vorkommt. Beim Menschen ist es in Brust, Darm, Knochenbälkchen der Substantia spongiosa und Fibroblasten lokalisiert. Es oxidiert, als Ferroxidase, das von Enterozyten importierte Eisen(II) zu Eisen(III) und bereitet es für den Export durch das Ferroportin vor. Hephästin ist damit zusammen mit dem Ferroportin für den Efflux von Eisenionen aus der Zelle verantwortlich. Damit ist es unverzichtbar im Eisen-Stoffwechsel bei der Eisenaufnahme durch den Darm. Möglicherweise transportiert es auch Kupfer.

Das Protein ist nach dem griechischen Gott Hephaistos (Schmied) benannt.<ref>{{#if: | | UniProt }} {{#if:|{{{titel}}}|Q9BQS7}}{{#if:|Vorlage:Abrufdatum}}</ref><ref>{{#invoke:Vorlage:Literatur|f}}{{#if: 

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Pathologie

Kupfer ist essenziell notwendig für die Stabilität von Hephästin. Mäuse ganz ohne Caeruloplasmin und Hephästin zeigen alle Symptome der Makuladegeneration. Erhöhte Eisenkonzentrationen in der Substantia nigra bei Parkinsonkrankheit sind wahrscheinlich auf erniedrigte Aktivität von Hephästin und Ferroportin zurückzuführen.<ref>{{#invoke:Vorlage:Literatur|f}}{{#if: 

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Regulation

Erythropoetin erhöht die Hephästinexpression und Eisenabsorption im Duodenum bzw. aus dem intestinalen Lumen. Eine eisenhaltige Diät erhöhte bei Ratten die Expression von Caeruloplasmin und Ferroportin, nicht jedoch von Hephästin. In Darmzellen ist der Übergang ins Krebsstadium mit einer erhöhten Eiseneinfuhr und vermindertem Export (Hephästinaktivität) verknüpft.<ref>{{#invoke:Vorlage:Literatur|f}}{{#if: 

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Datei:TEST Hephaestin illustration.png
Hypothetische Regulierung der Hephästinexpression (Hephästin= Hp) als Reaktion auf unterschiedliche Eisenaufnahme und -speicherung (Eisen-Stoffwechsel).

Einzelnachweise

<references />

Abgerufen von „https://wiki-de.moshellshocker.dns64.de/index.php?title=Hephästin&oldid=1433116“