GTX88689
GCH1 antibody, Internal
Eine Lieferung in diese Region ist nicht möglich.
- SKU:
- GTX88689
- Zusätzliche Namen:
- GTP cyclohydrolase 1 , DYT14 , DYT5 , DYT5a , GCH , GTP-CH-1 , GTPCH1 , HPABH4B
- Anwendung:
- WB
- Konzentration:
- 0.50 mg/ml
- Physischer Zustand:
- Liquid
- Spezies-Reaktivität:
- Human
- Aufreinigung:
- Ammonium Sulfate Precipitated; Affinity Purified
- Lagerbedingungen:
- -20[o]C Aliquot. Avoid freeze/thaw cycles. Store undiluted., 2-8[o]C Aliquot. Avoid freeze/thaw cycles. Store undiluted., -20[o]C/-70[o]C Aliquot. Avoid freeze/thaw cycles. Store undiluted.
- Hersteller:
- Genetex
- Host:
- Goat
- Reaktivitäten:
- Human
- Buffer:
- TBS, 0.5% BSA, 0.02% Sodium azide.
- Immunogen:
- Peptide with sequence C-GKVHIGYLPNKQ, from the internal region of the protein sequence according to NP_000152.1 ; NP_001019195.1 ; NP_001019241.1 ; NP_001019242.1 .
- Uniprot:
- P30793
- Synonyme:
- dystonia 14;DYT14;DYT5;DYT5a;GCH;GTP cyclohydrolase 1;GTP cyclohydrolase I;GTP-CH-1;GTP-CH-I;GTPCH1;guanosine 5'-triphosphate cyclohydrolase I;HPABH4B
- Weitere Details:
- 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, Jul 2008]
- Versandbedingungen:
- Blue Ice