This gene encodes a member of the sedolisin family of serine proteases. The protease functions in the lysosome to cleave N-terminal tripeptides from substrates, and has weaker endopeptidase activity. It is synthesized as a catalytically-inactive enzyme which is activated and auto-proteolyzed upon acidification. Mutations in this gene result in late-infantile neuronal ceroid lipofuscinosis, which is associated with the failure to degrade specific neuropeptides and a subunit of ATP synthase in the lysosome.
Caractéristiques techniques
| Marque | Invitrogen |
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| Réf. B-LABO | BL-RE-106529 |
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| Réf. fabricant | 18517914 |
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| Numéro d’adhésion | Cell growth-inhibiting gene 1 protein; ceroid-lipofuscinosis, neuronal 2; CLN 2; Cln2; GIG 1; GIG1; growth-inhibiting protein 1; LPIC; lysosomal pepstatin insensitive protease; Lysosomal pepstatin-insensitive protease; MGC21297; SCAR7; TPP 1; TPP I; TPP1; TPP-1; TPPI; TPP-I; tripeptidyl aminopeptidase; tripeptidyl peptidase 1; tripeptidyl peptidase I; tripeptidyl peptidase-I; tripeptidyl-peptidase 1; Tripeptidyl-peptidase I; UNQ267/PRO304 |
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| Plage de dosage | 10 to 2,500 pg/mL |
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| Sensibilité du dosage | 10 pg/mL |
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| Conjugué | Biotin |
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| Type de produit | ELISA |
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| Type d’échantillon | Plasma |
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| À utiliser avec (équipement) | Colorimetric Microplate Reader |
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| Identification génétique (Entrez) | O14773 |
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| Symbole de gène(s) | TPP1 |
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| Coefficient de variation inter-dosage | <12% |
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| Coefficient de variation intra-dosage | <10% |
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| Contenus des kits | Pre-coated 96 well plate, Standard, Assay Diluent concentrate, Biotinylated Detection Antibody, SAV-HRP, Wash Buffer, Chromogen, Stop Solution, Adhesive Plate Covers |
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| Marqueur ou colorant | HRP |
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| Quantité | 10 x 96 Tests |
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| État réglementaire | Research Use Only |
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| Volume d’échantillon | Serum, 50 µL; Supernatant, 50 µL; Plasma, 50 µL |
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| Conditions de stockage | 2°C to 8°C |
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| Cible | CLN2 |
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| Heure du test | 1 hr 20 min |
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| Durée totale du dosage | 4 hr 45 min |
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