Complement Factor I Antikörper (N-Term)
Kurzübersicht für Complement Factor I Antikörper (N-Term) (ABIN2789418)
Target
Alle Complement Factor I (CFI) Antikörper anzeigenReaktivität
Wirt
Klonalität
Konjugat
Applikation
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Bindungsspezifität
- N-Term
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Sequenz
- WSMREANVAC LDLGFQQGAD TQRRFKLSDL SINSTECLHV HCRGLETSLA
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Homologie
- Human: 100%
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Produktmerkmale
- This is a rabbit polyclonal antibody against CFI. It was validated on Western Blot.
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Aufreinigung
- Affinity Purified
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Applikationshinweise
- Optimal working dilutions should be determined experimentally by the investigator.
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Kommentare
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Antigen size: 583 AA
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Beschränkungen
- Nur für Forschungszwecke einsetzbar
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Format
- Liquid
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Konzentration
- Lot specific
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Buffer
- Liquid. Purified antibody supplied in 1x PBS buffer with 0.09 % (w/v) sodium azide and 2 % sucrose.
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Konservierungsmittel
- Sodium azide
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Vorsichtsmaßnahmen
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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Handhabung
- Avoid repeated freeze-thaw cycles.
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Lagerung
- -20 °C
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Informationen zur Lagerung
- For short term use, store at 2-8°C up to 1 week. For long term storage, store at -20°C in small aliquots to prevent freeze-thaw cycles.
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- Complement Factor I (CFI)
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Andere Bezeichnung
- CFI
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Hintergrund
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This gene encodes a serine proteinase that is essential for regulating the complement cascade. The encoded preproprotein is cleaved to produce both heavy and light chains, which are linked by disulfide bonds to form a heterodimeric glycoprotein. This heterodimer can cleave and inactivate the complement components C4b and C3b, and it prevents the assembly of the C3 and C5 convertase enzymes. Defects in this gene cause complement factor I deficiency, an autosomal recessive disease associated with a susceptibility to pyogenic infections. Mutations in this gene have been associated with a predisposition to atypical hemolytic uraemic syndrome, a disease characterized by acute renal failure, microangiopathic hemolytic anemia and thrombocytopenia. Primary glomerulonephritis with immmune deposits is another condition associated with mutation of this gene.
Alias Symbols: AHUS3, C3BINA, C3b-INA, FI, IF, KAF
Protein Interaction Partner: GLP1R, CFH, C3,
Protein Size: 583 -
Molekulargewicht
- 64 kDa
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Gen-ID
- 3426
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NCBI Accession
- NM_000204, NP_000195
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UniProt
- P05156
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Pathways
- Komplementsystem
Target
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