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- Antibody type:Primary
- Antigen name:Glycine cleavage system protein H (aminomethyl carrier)
- Antigen symbol:GCSH
- Clonality:Polyclonal
- Conjugation:Unconjugated
- Host:Rabbit
- ImmunoChemistry:Yes
- Isotype:IgG
- Reactivity:Human,Rat,Mouse
- Western blot:Yes
- 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 -20°C for 12 months.
- Concentration:1 ug/ul
- Shipping temperature:4 °C
- Purification:Purified by Protein A.
- Size:100 μg
- Pk:100 µl
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.
WB(1:100-1000), IHC-P(1:100-500), IF(IHC-P)(1:50-200)
Type: Primary
Antigen: GCSH
Clonality: Polyclonal
Clone:
Conjugation: Unconjugated
Epitope:
Host: Rabbit
Isotype: IgG
Reactivity: Human, Mouse, Rat