GCSH Antikörper (AA 101-173) (FITC)

Produktnummer ABIN1709514

Dieses Anti-GCSH-Antikörper ist ein Kaninchen Polyklonal-Antikörper zur Detektion von GCSH in WB, IF (cc) und IF (p). Geeignet für Human.

Kurzübersicht für GCSH Antikörper (AA 101-173) (FITC) (ABIN1709514)

Target: GCSH (Glycine Cleavage System H Protein (GCSH))

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser GCSH Antikörper ist konjugiert mit FITC

Applikation: Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))

Produktdetails anti-GCSH Antikörper (FITC)

Bindungsspezifität: AA 101-173

Homologie: Human,Mouse,Rat,Horse

Aufreinigung: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human GCSH

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

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

Details zu GCSH

Target: GCSH (Glycine Cleavage System H Protein (GCSH))

Andere Bezeichnung: GCSH

Hintergrund:

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