Anti-GCH1 antibody produced in rabbit
SIGMA/SAB1410516 - purified immunoglobulin, buffered aqueous solution
Synonym: DYT14; DYT5; GCH; GTP-CH-1; GTPCH1
Product Type: Chemical
Western Blotting Western Blot analysis of GCH1 expression in transfected 293T cell line by GCH1 polyclonal antibody. Lanes Lane 1: GCH1 transfected lysate (27.90 kDa). Lane 2: Non-transfected lysate.
| antibody form | purified immunoglobulin |
| antibody product type | primary antibodies |
| biological source | rabbit |
| clone | polyclonal |
| conjugate | unconjugated |
| form | buffered aqueous solution |
| mol wt | antigen 27.9 kDa |
| NCBI accession no. | NM_000161.2   |
| Quality Level | 100 |
| shipped in | dry ice |
| species reactivity | human |
| storage temp. | −20°C |
| target post-translational modification | unmodified |
| technique(s) | western blot: 1 μg/mL |
| UniProt accession no. | P30793 |
| General description: | This gene encodes a member of the GTP cyclohydrolase family. The encoded protein is the first and rate-limiting enzyme in tetrahydrobiopterin (BH4) biosynthesis, catalyzing the conversion of GTP into 7,8-dihydroneopterin triphosphate. BH4 is an essential cofactor required by aromatic amino acid hydroxylases as well as nitric oxide synthases. Mutations in this gene are associated with malignant hyperphenylalaninemia and dopa-responsive dystonia. Several alternatively spliced transcript variants encoding different isoforms have been described; however, not all variants give rise to a functional enzyme. (provided by RefSeq) |
| Immunogen: | GCH1 (NP_000152.1, 1 a.a. ~ 250 a.a) full-length human protein. Sequence MEKGPVRAPAEKPRGARCSNGFPER |
| Physical form: | Solution in phosphate buffered saline, pH 7.4 |
| RIDADR | NONH for all modes of transport |
| WGK Germany | WGK 3 |
| Flash Point(F) | Not applicable |
| Flash Point(C) | Not applicable |
| Storage Temp. | −20°C |
| UNSPSC | 12352203 |