GCSH Antikörper (AA 101-173) (AbBy Fluor® 647)
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- Target Alle GCSH Antikörper anzeigen
- GCSH (Glycine Cleavage System H Protein (GCSH))
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Bindungsspezifität
- AA 101-173
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Reaktivität
- Human
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Wirt
- Kaninchen
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Klonalität
- Polyklonal
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Konjugat
- Dieser GCSH 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))
- Homologie
- Human,Mouse,Rat,Horse
- Aufreinigung
- Purified by Protein A.
- Immunogen
- KLH conjugated synthetic peptide derived from human GCSH
- Isotyp
- IgG
- Top Product
- Discover our top product GCSH 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
- GCSH (Glycine Cleavage System H Protein (GCSH))
- Andere Bezeichnung
- GCSH (GCSH Produkte)
- Synonyme
- GCE antikoerper, NKH antikoerper, GCSH antikoerper, H-PROTEIN antikoerper, 1100001L02Rik antikoerper, 5730591C18Rik antikoerper, wu:fb14d10 antikoerper, wu:fw84b08 antikoerper, zgc:112535 antikoerper, BA5228 antikoerper, gce antikoerper, gcsh antikoerper, nkh antikoerper, wu:fd56h06 antikoerper, zgc:110557 antikoerper, zgc:92732 antikoerper, glycine cleavage system protein H antikoerper, glycine cleavage system protein H (aminomethyl carrier) antikoerper, glycine cleavage system lipoyl carrier protein GcvH antikoerper, glycine cleavage system H protein antikoerper, glycine cleavage system protein H (aminomethyl carrier), a antikoerper, glycine cleavage system carrier of aminomethyl moiety GcvH antikoerper, leucine rich repeat containing 34 antikoerper, glycine cleavage system protein H (aminomethyl carrier) L homeolog antikoerper, glycine cleavage system protein H (aminomethyl carrier), b antikoerper, GCSH antikoerper, Gcsh antikoerper, gcvH-2 antikoerper, gcvH antikoerper, gcsha antikoerper, LRRC34 antikoerper, gcsh.L antikoerper, gcshb antikoerper
- Hintergrund
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Synonyms: GCE, GCSH, GCSH_HUMAN, Glycine cleavage system H protein, Glycine cleavage system H protein mitochondrial, Glycine cleavage system protein H aminomethyl carrier, Glycine cleavage system protein H, Lipoic acid containing protein, mitochondrial, Mitochondrial glycine cleavage system H protein, NKH.
Background: GCSH is a 173 amino acid mitochondrial protein that contains one lipoyl-binding domain and belongs to the gcvH family. Defects in the gene encoding GCSH are the cause of glycine encephalopathy (GCE), an autosomal recessive disease that is also referred to as non-ketotic hyperglycinemia (NKH). Characterized by severe neurological symptoms, patients with GCE have a large amount of glycine accumulated in their body fluids. The gene encoding GCSH maps to human chromosome 16, which encodes over 900 genes and comprises nearly 3 % of the human genome.
- Gen-ID
- 2653
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