Monoclonal Anti-GCH1 antibody produced in mouse
SIGMA/WH0002643M1 - clone 4A12, purified immunoglobulin, buffered aqueous solution
Synonym: Anti-DYT5; Anti-GCH; Anti-GTP cyclohydrolase 1 (dopa-responsive dystonia); Anti-GTPCH1
Product Type: Chemical
Immunohistochemistry Immunoperoxidase of monoclonal antibody to GCH1 on formalin-fixed paraffin-embedded human lymph node. [antibody concentration 3 μg/mL]
Western Blotting GCH1 monoclonal antibody, clone 4A12 Western Blot analysis of GCH1 expression in IMR-32.
Western Blotting Western Blot analysis of GCH1 expression in transfected 293T cell line by GCH1 monoclonal antibody, clone 4A12. Lanes Lane 1: GCH1 transfected lysate (27.9 kDa). Lane 2: Non-transfected lysate.
Western Blotting QC Western Blot detection against Immunogen (35.53 kDa).
Immunoprecipitation Immunoprecipitation of GCH1 transfected lysate using anti-GCH1 monoclonal antibody and Protein A Magnetic Bead , and immunoblotted with GCH1 rabbit polyclonal antibody.
ELISA Detection limit for recombinant GST tagged GCH1 is approximately 0.3 ng/mL as a capture antibody.
| antibody form | purified immunoglobulin |
| antibody product type | primary antibodies |
| biological source | mouse |
| clone | 4A12, monoclonal |
| conjugate | unconjugated |
| form | buffered aqueous solution |
| GenBank® accession no. | NM_000161   |
| isotype | IgG2aκ |
| Quality Level | 100 |
| shipped in | dry ice |
| species reactivity | human |
| storage temp. | −20°C |
| technique(s) | immunohistochemistry (formalin-fixed, paraffin-embedded sections): suitable |
| immunoprecipitation (IP): suitable | |
| indirect ELISA: suitable | |
| western blot: 1-5 μg/mL | |
| UniProt accession no. | P30793 |
| Biochem/physiol Actions: | Guanosine triphosphate (GTP) cyclohydrolase I (GCH1) catalyzes the synthesis of 7,8-dihydroneopterin triphosphate from GTP. It acts as a rate-limiting enzyme in the tetrahydrobiopterin (BH4) biosynthesis pathway. GCH1 requires zinc (Zn2+) for its catalytic function. It is crucial for dopamine synthesis, and its variants may have a role in the pathogenesis of Parkinson′s disease (PD). Mutations in the GCH1 gene are implicated in Segawa disease, hyperphenylalaninemia, and dopa-responsive dystonia (DRD). GCH1 gene polymorphisms are correlated to the pathophysiology of obstructive sleep apnea (OSA) and in neuropathic pain and stroke. |
| Disclaimer: | Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals. |
| General description: | Guanosine triphosphate (GTP) cyclohydrolase I (GCH1), a homodecameric protein belongs to the GTP cyclohydrolase family. Many isoforms of GCH1 are present due to alternative splicing. However, not all variants encode a functional enzyme. The GCH1 gene is mapped to human chromosome 14q22.2. |
| Immunogen: | GCH1 (NP_000152, 84 a.a. ~ 172 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa. Sequence ENPQRQGLLKTPWRAASAMQFFTKG |
| Legal Information: | GenBank is a registered trademark of United States Department of Health and Human Services |
| Physical form: | Solution in phosphate buffered saline, pH 7.4 |
| RIDADR | NONH for all modes of transport |
| Flash Point(F) | Not applicable |
| Flash Point(C) | Not applicable |
| Storage Temp. | −20°C |
| UNSPSC | 12352203 |