CLN8 Antikörper (AA 201-286) (AbBy Fluor® 647)
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- Target Alle CLN8 Antikörper anzeigen
- CLN8 (Ceroid-Lipofuscinosis, Neuronal 8 (Epilepsy, Progressive with Mental Retardation) (CLN8))
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Bindungsspezifität
- AA 201-286
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Reaktivität
- Human, Ratte
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Wirt
- Kaninchen
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Klonalität
- Polyklonal
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Konjugat
- Dieser CLN8 Antikörper ist konjugiert mit AbBy Fluor® 647
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Applikation
- Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
- Kreuzreaktivität
- Human, Ratte
- Homologie
- Mouse,Dog,Pig,Horse,Rabbit
- Aufreinigung
- Purified by Protein A.
- Immunogen
- KLH conjugated synthetic peptide derived from human CLN8
- Isotyp
- IgG
- Top Product
- Discover our top product CLN8 Primärantikörper
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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
- Konzentration
- 1 μg/μL
- Buffer
- Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
- Konservierungsmittel
- ProClin
- Vorsichtsmaßnahmen
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
- Lagerung
- -20 °C
- Informationen zur Lagerung
- Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
- Haltbarkeit
- 12 months
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- Target
- CLN8 (Ceroid-Lipofuscinosis, Neuronal 8 (Epilepsy, Progressive with Mental Retardation) (CLN8))
- Andere Bezeichnung
- CLN8 (CLN8 Produkte)
- Synonyme
- mnd antikoerper, C8orf61 antikoerper, EPMR antikoerper, CLN8, transmembrane ER and ERGIC protein antikoerper, ceroid-lipofuscinosis, neuronal 8 antikoerper, CLN8 antikoerper, Cln8 antikoerper
- Hintergrund
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Synonyms: Ceroid-lipofuscinosis, neuronal 8 epilepsy, progressive with mental retardation, Cln8, CLN8_HUMAN, EPMR, Protein CLN8, C8orf61
Background: CLN8, a 286 amino acid transmembrane protein, localizes mainly to the endoplasmic reticulum, but also partially to the ER-Golgi intermediate compartment (ERGIC). Mutations in the CLN8 gene cause neuronal ceroid lipofuscinosis 8 and progressive epilepsy with mental retardation (EPMR). Both disorders are forms of neuronal ceroid-lipofuscinose (NCL), a group of progressive neurodegenerative diseases found in children, characterized by failure of psychomotor development, impaired vision, seizures and premature death. The CLN8 protein is one of eight proteins in the CLN family, including CLN1-CLN7, which are associated with NCL.
- Gen-ID
- 2055
- UniProt
- Q9UBY8
- Pathways
- Regulation of Cell Size, Dicarboxylic Acid Transport
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