Fibrillin 2 Antikörper (AA 1001-1200) (Cy7)
Kurzübersicht für Fibrillin 2 Antikörper (AA 1001-1200) (Cy7) (ABIN1413432)
Target
Alle Fibrillin 2 (FBN2) Antikörper anzeigenReaktivität
Wirt
Klonalität
Konjugat
Applikation
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Bindungsspezifität
- AA 1001-1200
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Homologie
- Human,Mouse,Rat,Dog,Cow,Sheep,Pig,Horse,Chicken,Rabbit
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Aufreinigung
- Purified by Protein A.
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Immunogen
- KLH conjugated synthetic peptide derived from human Fibrillin 2
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Isotyp
- IgG
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Applikationshinweise
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IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200 -
Beschränkungen
- Nur für Forschungszwecke einsetzbar
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Format
- Liquid
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Konzentration
- 1 μg/μL
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Buffer
- Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
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Konservierungsmittel
- ProClin
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Vorsichtsmaßnahmen
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
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Lagerung
- -20 °C
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Informationen zur Lagerung
- Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
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Haltbarkeit
- 12 months
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- Fibrillin 2 (FBN2)
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Andere Bezeichnung
- Fibrillin 2
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Hintergrund
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Synonyms: CCA, congenital contractural arachnodactyly Marfanoid-like, DA9, FBN2, FBN2_HUMAN, fibrillin 2 congenital contractural arachnodactyly, Fibrillin-2.
Background: Extracellular glycoproteins fibrillin-1 and -2 are major components of connective tissue microfibrils. Fibrillin-2 containing microfibrils regulate the early process of elastic fiber assembly in tissue. Mutations in the fibrillin-2 gene resulting in impaired assembly of fibrillin-2 may lead to molecular congenital contractural arachnodactyly. Fibrillin-2 constitutes the backbone of microfibrils which insert directly into the lamina densa of basement membranes. Epithelial cells primarily deposit fibrillin into the extracellular matrix in a nonfibrillar form. Mutations in the 8-cysteine motif of Fibrillin-2 alters its binding to microfibril-associated glycoprotein-1 (MAGP-1), which may increase the severity of congenital contractural arachnodactyly.
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Pathways
- Maintenance of Protein Location, SARS-CoV-2 Protein Interaktom
Target
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