-
Product Name
Anti-GCSH antibody
- Documents
-
Description
Rabbit polyclonal to GCSH
-
Tested applications
ELISA, IHC-P
-
Species reactivity
Human GCSH
-
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
- Immunogen
-
Isotype
Rabbit IgG
-
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.
-
Clonality
Polyclonal
-
Formulation
0.2 μm filtered solution in PBS
-
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
-
Validations
GCSH Antibody, Rabbit PAb, Antigen Affinity Purified, Immunohistochemistry
GCSH Antibody, Rabbit PAb, Antigen Affinity Purified, Immunohistochemistry
-
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.
-
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.
Related Products / Services
Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"