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Specifications
- Antibody type:Primary
- Antigen name:Glycine Ceavage System Protein H (aminomethyl carrier)
- Antigen symbol:GCSH
- Clonality:Polyclonal
- Conjugation:Alexa Fluor® 350
- Host:Rabbit
- ImmunoChemistry:Yes
- Isotype:IgG
- Reactivity:Human,Rat,Mouse
- Cross adsorption:No
- Form:liquid
- Gene ID:2653
- Antigen synonyms:NKH|GCE
- Storage buffer:Aqueous buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% sodium azide. Store at 4°C for 12 months.
- Storage temperature:Store at 4°C for 12 months
- Concentration:1 μg/μl
- Shipping temperature:4°C
- Purification:Purified by Protein A
- Pk:100 µl
Specifications
About this item
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.
Recommended Dilutions: IF(IHC-P): 1:50-200
Type: Primary
Antigen: GCSH
Clonality: Polyclonal
Clone:
Conjugation: Alexa Fluor® 350
Epitope:
Host: Rabbit
Isotype: IgG
Reactivity: Human, Mouse, Rat