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Proteinase 3

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{{#if: | {{#if: 1fuj| {{#if:9495| {{#if: 221 aa; 24,2 kDa | {{#if: | {{#if: | {{#if: | {{#if: | {{#if: PRTN3PR3, ACPA, AGP7, C-ANCA, MBT, P29, PR-3P24158177020893580 | {{#if: PRTN3PR3, ACPA, AGP7, C-ANCA, MBT, P29, PR-3 | {{#if: P24158177020893580 | {{#if: | {{#if:| {{#if: | {{#if: 3.4.21.76SerinproteaseS01.134Hydrolyse von Proteinen incl. ElastinProtein mit Aminosäure -Ala-+-Xaa- oder -Val-+-Xaa-Spaltprodukte | {{#if: 3.4.21.76Serinprotease| {{#if: S01.134| {{#if: | {{#if: Hydrolyse von Proteinen incl. Elastin| {{#if: Protein mit Aminosäure -Ala-+-Xaa- oder -Val-+-Xaa-| {{#if: Spaltprodukte| {{#if: Lebewesen{{#if: P24158 | {{#ifeq: Trypsin Precursor | NV ||1}}|}}| {{#if: P24158 | {{#ifeq: Trypsin Precursor | NV || | style="background:#C3FDB8; color:#000000;" | Homologie-Familie {{#if: Lebewesen | | style="background:#C3FDB8; color:#000000;" | Übergeordnetes Taxon {{#if: | | style="background:#C3FDB8; color:#000000;" | Ausnahmen {{#if: {{#if: Maus | | colspan="3" style="background:#90EE90; color:#202122; text-align:center;" | Orthologe {{#if: ENSG00000196415ENSMUSG00000057729
{{#if: Proteinase 3 | Proteinase 3 | Proteinase 3 }}
Proteinase 3 }}
nach PDB 1FUJ
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Andere Namen

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Vorhandene Strukturdaten: 1fuj }}

Eigenschaften des menschlichen Proteins

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Masse/Länge Primärstruktur 221 aa; 24,2 kDa

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Sekundär- bis Quartärstruktur

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Kofaktor

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Präkursor

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Isoformen

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Bezeichner
{{#if:PR3, ACPA, AGP7, C-ANCA, MBT, P29, PR-3 | Gen-Namen | Gen-Name}} | Gen-Name(n) }} {{#if:9495 | PRTN3 | PRTN3}}{{#if:PR3, ACPA, AGP7, C-ANCA, MBT, P29, PR-3 |, PR3, ACPA, AGP7, C-ANCA, MBT, P29, PR-3}} }}
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=

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}}

Arzneistoffangaben
ATC-Code 
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 {{#if:|
DrugBank [1] 
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 {{#if:|
Wirkstoffklasse 
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Transporter-Klassifikation
TCDB {{{TCDB}}} 
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Bezeichnung {{{TranspText}}} 
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}}

{{#if:|Inhibitorklassifikation|{{#if:|Enzymklassifikationen|Enzymklassifikation}}}}
EC, Kategorie {{#if:3.4.21.76| 3.4.21.76}}{{#if: Serinprotease|, Serinprotease}} }}
MEROPS S01.134}}
MEROPS {{{Inhibitor_fam}}}}}
Reaktionsart Hydrolyse von Proteinen incl. Elastin 
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Substrat Protein mit Aminosäure -Ala-+-Xaa- oder -Val-+-Xaa- 
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Produkte Spaltprodukte 
 }}


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Vorkommen
{{#if: Trypsin Precursor|Hovergen}}] Trypsin Precursor|Hovergen}}]}} }} }}
Lebewesen }}
}}

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Mensch Maus
Entrez 
 {{ #if: 5657
5657 }} 
 {{ #if: 19152
19152 }}
style="background:#C3FDB8; color:#202122;" | Ensembl 
    {{ #if: ENSG00000196415
ENSG00000196415 }} 
    {{ #if: ENSMUSG00000057729
ENSMUSG00000057729 }}

} 

 |-
 | style="background:#C3FDB8; color:#202122;" | UniProt
 {{ #if: P24158
    | | {{#if: kurz| | UniProt }} {{#if:|{{{titel}}}|P24158}}{{#if:|Vorlage:Abrufdatum}}
    | |  }}
 {{ #if: Q61096
    | | {{#if: kurz| | UniProt }} {{#if:|{{{titel}}}|Q61096}}{{#if:|Vorlage:Abrufdatum}}
    | |  }}
 |-
 {{#if: NM_002777NM_011178
    | | style="background:#C3FDB8; color:#202122;" | Refseq (mRNA)
      {{ #if: NM_002777
         | | NM_002777
         | |  }}
      {{ #if: NM_011178
         | | NM_011178
         | |  }}
    |}}
 |-
 {{#if: NP_002768NP_035308
    | | style="background:#C3FDB8; color:#202122;" | Refseq (Protein)
      {{ #if: NP_002768
         | | NP_002768
         | |  }}
      {{ #if: NP_035308
         | | NP_035308
         | |  }}
    |}}
 |-
 {{#if: 19791985799175107928279679286303
    | | style="background:#C3FDB8; color:#202122;" | Genlocus
      {{#if: 19 
        | {{#if: 791985 
            | {{#if: 799175 
                | |  #if:  |?db=|}}&position=chr19:791985-799175 Chr 19: {{#expr: 791985 / 1000000 round 2}} – {{#expr: 799175 / 1000000 round 2}} Mb 
                | |  }}
            | |  }}
        | |  }}
      {{#if: 10 
        | {{#if: 79282796 
            | {{#if: 79286303 
                | |  #if:  |?db=|}}&position=chr10:79282796-79286303 Chr 10: {{#expr: 79282796 / 1000000 round 2}} – {{#expr: 79286303 / 1000000 round 2}} Mb 
                | |  }}
           | |  }}
       | |  }}
    |}}
 |-
 | style="background:#C3FDB8; color:#202122;" | PubMed-Suche 
 {{ #if: 5657
    | | 5657
    | |  }}
 {{ #if: 19152
    | | 19152
    | |  }}

| |- | colspan="3" style="background:#90EE90; color:#202122; text-align:center;" | Orthologe (Mensch) |- | style="background:#C3FDB8; color:#202122;" | Entrez | colspan="2" | 5657 

 {{#if: ENSG00000196415
    | |-

| style="background:#C3FDB8; color:#202122;" | Ensembl | colspan="2" | ENSG00000196415 }} |- | style="background:#C3FDB8; color:#202122;" | UniProt | colspan="2" | {{#if: kurz| | UniProt }} {{#if:|{{{titel}}}|P24158}}{{#if:|Vorlage:Abrufdatum}} 

 {{#if: NM_002777
    | |-

| style="background:#C3FDB8; color:#202122;" | Refseq (mRNA) | colspan="2" | NM_002777 }} 

 {{#if: NP_002768
    | |-

| style="background:#C3FDB8; color:#202122;" | Refseq (Protein) | colspan="2" | NP_002768 }} 

 {{#if: 19 
   | {{#if: 791985 
       | {{#if: 799175 
           | |-

| style="background:#C3FDB8; color:#202122;" | Genlocus | colspan="2" | #if: |?db=|}}&position=chr19:791985-799175 Chr 19: {{#expr: 791985 / 1000000 round 2}} – {{#expr: 799175 / 1000000 round 2}} Mb 

           |  }}
       |  }}
   |  }}

|- | style="background:#C3FDB8; color:#202122;" | PubMed-Suche | colspan="2" | 5657 }} {{#if: Maus 

    | {{#if: 19152ENSMUSG00000057729NM_011178NP_035308107928279679286303Q61096 
        |
        |  Spezies2= ist definiert, es sind jedoch keine weiteren Angaben zu Spezies2 vorhanden.{{#ifeq:0|0| }} }}
    | {{#if: 19152ENSMUSG00000057729NM_011178NP_035308107928279679286303Q61096 
        |  Es sind Angaben zu Spezies2 vorhanden, Spezies2= ist jedoch nicht definiert.{{#ifeq:0|0| }}
        |}}

}} | {{#if: Maus | | 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;" | Maus
 |-
 | style="background:#C3FDB8; color:#202122;" | Entrez
 {{ #if: 5657
    | | 5657
    | |  }}
 {{ #if: 19152
    | | 19152
    | |  }}
 |-
 {{#if: ENSG00000196415ENSMUSG00000057729
    | | style="background:#C3FDB8; color:#202122;" | Ensembl
    {{ #if: ENSG00000196415
       || ENSG00000196415
       ||  }}
    {{ #if: ENSMUSG00000057729
       || ENSMUSG00000057729
       ||  }}
  |}}
 |-
 | style="background:#C3FDB8; color:#202122;" | UniProt
 {{ #if: P24158
    | | {{#if: kurz| | UniProt }} {{#if:|{{{titel}}}|P24158}}{{#if:|Vorlage:Abrufdatum}}
    | |  }}
 {{ #if: Q61096
    | | {{#if: kurz| | UniProt }} {{#if:|{{{titel}}}|Q61096}}{{#if:|Vorlage:Abrufdatum}}
    | |  }}
 |-
 {{#if: NM_002777NM_011178
    | | style="background:#C3FDB8; color:#202122;" | Refseq (mRNA)
      {{ #if: NM_002777
         | | NM_002777
         | |  }}
      {{ #if: NM_011178
         | | NM_011178
         | |  }}
    |}}
 |-
 {{#if: NP_002768NP_035308
    | | style="background:#C3FDB8; color:#202122;" | Refseq (Protein)
      {{ #if: NP_002768
         | | NP_002768
         | |  }}
      {{ #if: NP_035308
         | | NP_035308
         | |  }}
    |}}
 |-
 {{#if: 19791985799175107928279679286303
    | | style="background:#C3FDB8; color:#202122;" | Genlocus
      {{#if: 19 
        | {{#if: 791985 
            | {{#if: 799175 
                | |  #if:  |?db=|}}&position=chr19:791985-799175 Chr 19: {{#expr: 791985 / 1000000 round 2}} – {{#expr: 799175 / 1000000 round 2}} Mb 
                | |  }}
            | |  }}
        | |  }}
      {{#if: 10 
        | {{#if: 79282796 
            | {{#if: 79286303 
                | |  #if:  |?db=|}}&position=chr10:79282796-79286303 Chr 10: {{#expr: 79282796 / 1000000 round 2}} – {{#expr: 79286303 / 1000000 round 2}} Mb 
                | |  }}
           | |  }}
       | |  }}
    |}}
 |-
 | style="background:#C3FDB8; color:#202122;" | PubMed-Suche 
 {{ #if: 5657
    | | 5657
    | |  }}
 {{ #if: 19152
    | | 19152
    | |  }}

| |- | colspan="3" style="background:#90EE90; color:#202122; text-align:center;" | Orthologe (Mensch) |- | style="background:#C3FDB8; color:#202122;" | Entrez | colspan="2" | 5657 

 {{#if: ENSG00000196415
    | |-

| style="background:#C3FDB8; color:#202122;" | Ensembl | colspan="2" | ENSG00000196415 }} |- | style="background:#C3FDB8; color:#202122;" | UniProt | colspan="2" | {{#if: kurz| | UniProt }} {{#if:|{{{titel}}}|P24158}}{{#if:|Vorlage:Abrufdatum}} 

 {{#if: NM_002777
    | |-

| style="background:#C3FDB8; color:#202122;" | Refseq (mRNA) | colspan="2" | NM_002777 }} 

 {{#if: NP_002768
    | |-

| style="background:#C3FDB8; color:#202122;" | Refseq (Protein) | colspan="2" | NP_002768 }} 

 {{#if: 19 
   | {{#if: 791985 
       | {{#if: 799175 
           | |-

| style="background:#C3FDB8; color:#202122;" | Genlocus | colspan="2" | #if: |?db=|}}&position=chr19:791985-799175 Chr 19: {{#expr: 791985 / 1000000 round 2}} – {{#expr: 799175 / 1000000 round 2}} Mb 

           |  }}
       |  }}
   |  }}

|- | style="background:#C3FDB8; color:#202122;" | PubMed-Suche | colspan="2" | 5657 }} {{#if: Maus 

    | {{#if: 19152ENSMUSG00000057729NM_011178NP_035308107928279679286303Q61096 
        |
        |  Spezies2= ist definiert, es sind jedoch keine weiteren Angaben zu Spezies2 vorhanden.{{#ifeq:0|0| }} }}
    | {{#if: 19152ENSMUSG00000057729NM_011178NP_035308107928279679286303Q61096 
        |  Es sind Angaben zu Spezies2 vorhanden, Spezies2= ist jedoch nicht definiert.{{#ifeq:0|0| }}
        |}}

}} }}

|}{{#if:PBB_Protein_PRTN3_image.jpg||}}

Proteinase 3 (PR3, Myeloblastin) ist ein Enzym in Fischen und Vierbeinern (Euteleostomi, zu denen taxonomisch auch der Mensch gehört), das mit seiner antimikrobiellen Wirkung eine Rolle im Immunsystem spielt. Es handelt sich um eine Serinprotease, die mit der neutrophilen Elastase (ELA2) und Azurocidin (AZU) verwandt ist, auf demselben Genabschnitt kodiert ist und mit ihnen zusammen in neutrophile Granulozyten eingebaut wird. Bei einer Autoimmunerkrankung im Menschen, der Granulomatose mit Polyangiitis, werden Antikörper gegen das Enzym gebildet.<ref>{{#ifeq: | kurz | 177020 | Proteinase 3. }}{{#ifeq: | kurz | | In: {{#invoke:Vorlage:lang|flat}}. (englisch)}}.</ref>

Synthese

Das PR3-Gen auf Chromosom 19 ist über 6,5 Kilobasen und 5 Exons verteilt. Das Transkript hat eine Länge von 1001 Basen und kodiert für 256 Aminosäuren, welche nach Spleißen der Signalsequenz und des Propeptids ein Protein mit 221 Aminosäuren und 24 kDa Molmasse ergeben. Durch N-Glykosylierung an den Aminosäuren 129 und 174 (gerechnet vom ungespleißten Protein) entsteht schließlich die 29 kDa schwere Proteinase 3, ein kationisches Glykoprotein.<ref>Ensembl-Eintrag</ref><ref>{{#if: | | UniProt }} {{#if:|{{{titel}}}|P24158}}{{#if:|Vorlage:Abrufdatum}}.</ref>

Biologische Funktion

Proteinase 3 ist Bestandteil der primären (azurophilen, α-) Granula der neutrophilen Granulozyten und Lysosomen der Monozyten. Das Enzym kann bereits in frühen Phasen der Entwicklung von Monozyten und Granulozyten nachgewiesen werden. Werden Granulozyten aktiviert, verschmelzen die α-Granula mit der Zellmembran und setzen so Proteinase 3 frei. In vitro kann dieser Effekt durch TNF-α ausgelöst werden. Proteinase 3 hat proteolytische Eigenschaften und spaltet z. B. Elastin, Fibronectin, Laminin, Hämoglobin und Kollagen Typ IV. Proteinase 3 wirkt antimikrobiell gegen E. coli und C. albicans und ist an der Reifung myeloider Zellen beteiligt. Der natürliche Inhibitor von Proteinase 3 ist α-1-Antitrypsin.<ref>{{#invoke:Vorlage:Literatur|f}}{{#if: 

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Literatur

  • Lothar Thomas: Labor und Diagnose 5. Auflage, Frankfurt/Main 2000, ISBN 3-98052155-9

Einzelnachweise

<references />

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