GNS Antikörper (AA 1-100) (Cy7)
Kurzübersicht für GNS Antikörper (AA 1-100) (Cy7) (ABIN1708272)
Target
Alle GNS Antikörper anzeigenReaktivität
Wirt
Klonalität
Konjugat
Applikation
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Bindungsspezifität
 - AA 1-100
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Homologie
 - Human,Mouse,Rat,Cow,Horse,Rabbit
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Aufreinigung
 - Purified by Protein A.
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Immunogen
 - KLH conjugated synthetic peptide derived from human GNS/Glucosamine 6 sulfatase
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Isotyp
 - IgG
 
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Applikationshinweise
 - 
                        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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- GNS (Glucosamine (N-Acetyl)-6-Sulfatase (GNS))
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Andere Bezeichnung
 - Glucosamine 6 sulfatase
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Hintergrund
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Synonyms: 2610016K11Rik, AU042285, C87209, G6S, Glucosamine N-acetyl 6 sulfatase, Glucosamine 6 sulfatase, Glucosamine-6-sulfatase, GNS, GNS_HUMAN, MGC21274, N acetylglucosamine 6 sulfatase [Precursor], N-acetylglucosamine-6-sulfatase, N28088.
Background: GNS is a 552 amino acid lysosomal enzyme that hydrolyzes the 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate and heparan sulfate. A member of the sulfatase family, GNS assists in the catabolism of heparin, and binds calcium as a cofactor. GNS deficiency results in an autosomal recessive lysosomal storage disorder known as mucopolysaccharidosis type IIID (Sanfilippo D syndrome), which is characterized by mild somatic disease and severe degeneration of the central nervous system. Subject to post-translational internal peptidase cleavage, GNS is encoded by a gene mapping to human chromosome 12q14.2 and mouse chromosome 10 D2.
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Gen-ID
 - 2799
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Pathways
 - Glycosaminoglycan Metabolic Process
 
Target
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