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Product Name
Anti-GCSH antibody
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Description
Rabbit polyclonal to GCSH
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Tested applications
ELISA, IHC-P
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Species reactivity
Human GCSH
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Alternative names
glycine cleavage system protein H (aminomethyl carrier) antibody; GCSH antibody; GCE antibody; NKH antibody; glycine cleavage system H protein antibody; mitochondrial antibody; lipoic acid-containing protein antibody; mitochondrial glycine cleavage system H-protein antibody; GCE antibody; NKH antibody; glycine cleavage system protein H (aminomethyl carrier) antibody; Gcsh antibody; 1100001L02Rik antibody; 5730591C18Rik antibody; glycine cleavage system H protein antibody; mitochondrial antibody; 1100001L02Rik antibody; 5730591C18Rik antibody
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Isotype
Rabbit IgG
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Preparation
Produced in rabbits immunized with purified, recombinant Human GCSH (rh GCSH; AAA36011.1; Ser49-Glu173). GCSH specific IgG was purified by Human GCSH affinity chromatography.
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Clonality
Polyclonal
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Formulation
0.2 μm filtered solution in PBS
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Storage instructions
This antibody can be stored at 2℃-8℃ for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20℃ to -80℃. Preservative-Free.
Sodium azide is recommended to avoid contamination (final concentration 0.05%-0.1%). It is toxic to cells and should be disposed of properly. Avoid repeated freeze-thaw cycles. -
Applications
ELISA:0.1-0.2 μg/mL
This antibody can be used at 0.1-0.2 μg/mL with the appropriate secondary reagents to detect Human GCSH. The detection limit for Human GCSH is < 0.039 ng/well.
IHC-P: 0.1-2 μg/ml
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Background
Degradation of glycine is brought about by the glycine cleavage system, which is composed of four mitochondrial protein components: P protein (a pyridoxal phosphate-dependent glycine decarboxylase), H protein (a lipoic acid-containing protein), T protein (a tetrahydrofolate-requiring enzyme), and L protein (a lipoamide dehydrogenase). GCSH is the H protein, which transfers the methylamine group of glycine from the P protein to the T protein. Defects in GCSH gene are a cause of nonketotic hyperglycinemia (NKH). Two transcript variants, one protein-coding and the other probably not protein-coding,have been found for GCSH gene. Also, several transcribed and non-transcribed pseudogenes of GCSH gene exist throughout the genome.
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References
- Hiraga K. et al., 1988, Biochem Biophys Res Commun. 151 (2): 758-62.
- Fujiwara K. et al., 1991, Biochem Biophys Res Commun. 176 (2): 711-6.
- Koyata H. et al., 1991, Am J Hum Genet. 48 (2): 351-61.
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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"