GCSH Antikörper (AA 101-173)

Produktnummer ABIN1713812

Produktdetails anti-GCSH Antikörper

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

Bindungsspezifität: AA 101-173

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser GCSH Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), ELISA, Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunohistochemistry (Frozen Sections) (IHC (fro)), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunocytochemistry (ICC)

Homologie: Human,Mouse,Rat,Horse

Aufreinigung: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human GCSH

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: WB 1:300-5000
ELISA 1:500-1000
IHC-P 1:200-400
IHC-F 1:100-500
IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200
ICC 1:100-500

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 μg/μL

Buffer: 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % 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: 4 °C,-20 °C

Informationen zur Lagerung: Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.

Haltbarkeit: 12 months

Details zu GCSH

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:

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