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GCSH Polyclonal Antibody, Cy3 Conjugated

Product Specifications

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.

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.

Gene ID

2653

Cellular Locus

Cytoplasm

Host

Rabbit

Immunogen

101-173/173

Target

GCSH

Clonality

Polyclonal

Isotype

IgG

Conjugation

Cy3

Source

KLH conjugated synthetic peptide derived from human GCSH

Applications

WB, IF (IHC-P), IF (IHC-F), IF (ICC)

Purification

Purified by Protein A.

Excitation Emission

512,550nm/570,615nm

Concentration

1µg/µl

Dilution

WB (1:300-5000), IF (IHC-P) (1:50-200), IF (IHC-F) (1:50-200), IF (ICC) (1:50-200)

Buffer

Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Modification

Unmodified

Storage Conditions

Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Gene ID URL

2653

Predicted Cross Reactivity

Human, Mouse, Rat, Horse

Curated Selection

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