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The antibody against GCSH was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 1-173 of human GCSH (NP_004474.2) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, IF/ICC, ELISA.
The antibody against GCSH was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 1-173 of human GCSH (NP_004474.2) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, IF/ICC, ELISA.
| Cat.No | ADA-10285A | Clonality | Polyclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | GCSH |
| Target Synonyms | GCE; NKH; GCSH | Form | Liquid |
| Species Reactivity | Human, Mouse, Rat | Isotype | IgG |
| Storage Buffer | 50% Glycerol, PBS with 0.01% thimerosal, pH7.3. | Purification Method | Affinity purification |
| Positive Samples | Mouse kidney, Rat kidney | Application | ELISA, WB, IF/ICC |
| Immunogen Description | Recombinant fusion protein containing a sequence corresponding to amino acids 1-173 of human GCSH (NP_004474.2). | Target Species | Human |
|---|---|---|---|
| Immunogen Sequence | MALRVVRSVRALLCTLRAVPSPAAPCPPRPWQLGVGAVRTLRTGPALLSVRKFTEKHEWVTTENGIGTVGISNFAQEALGDVVYCSLPEVGTKLNKQDEFGALESVKAASELYSPLSGEVTEINEALAENPGLVNKSCYEDGWLIKMTLSNPSELDELMSEEAYEKYIKSIEE | Uniprot ID | P23434 |
Uniprot Id
P23434
Target Species
Human
Target Name
GCSH
Target Full Name
Glycine cleavage system H protein, mitochondrial
Target Function
The glycine cleavage system catalyzes the degradation of glycine. The H protein (GCSH) shuttles the methylamine group of glycine from the P protein (GLDC) to the T protein (GCST).
Target Involvement
Non-ketotic hyperglycinemia (NKH)
Target Subcellular Location
Mitochondrion.
Target Protein Families
GcvH family
Target Synonyms
GCSHGlycine cleavage system H protein; mitochondrial; Lipoic acid-containing protein
Target 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). The protein encoded by this gene is the H protein, which transfers the methylamine group of glycine from the P protein to the T protein. Defects in this 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 this gene. Also, several transcribed and non-transcribed pseudogenes of this gene exist throughout the genome.
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