SPR Antibody (PACO12441)
- SKU:
- PACO12441
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Application:
- IF
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
抗体名: | SPR Antibody (PACO12441) |
抗体コード: | PACO12441 |
サイズ: | 50ul |
宿主種: | Rabbit |
申し込み: | ELISA, WB, IF |
推奨される希釈: | |
反応性: | Human, Mouse, Rat |
免疫原: | Human SPR |
憲法: | Liquid |
ストレージバッファ: | PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles. |
精製方法: | Antigen Affinity purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | Non-conjugated |
シノニム: | sepiapterin reductase (7,8-dihydrobiopterin:NADP+ oxidoreductase);SPR;SDR38C1 ; |
UniProt Protein Function: | SPR: Catalyzes the final one or two reductions in tetra- hydrobiopterin biosynthesis to form 5,6,7,8-tetrahydrobiopterin. Defects in SPR are the cause of dystonia DOPA-responsive due to sepiapterin reductase deficiency (DRDSPRD). In the majority of cases, patients manifest progressive psychomotor retardation, dystonia and spasticity. Cognitive anomalies are also often present. The disease is due to severe dopamine and serotonin deficiencies in the central nervous system caused by a defect in BH4 synthesis. Dystonia is defined by the presence of sustained involuntary muscle contractions, often leading to abnormal postures. Belongs to the sepiapterin reductase family. |
UniProt Protein Details: | Protein type:Cofactor and Vitamin Metabolism - folate biosynthesis; EC 1.1.1.153; Oxidoreductase Chromosomal Location of Human Ortholog: 2p13.2 Cellular Component: cytosol; nucleoplasm Molecular Function:aldo-keto reductase activity; NADP binding; sepiapterin reductase activity Biological Process: nitric oxide biosynthetic process; regulation of nitric-oxide synthase activity; tetrahydrobiopterin biosynthetic process Disease: Dystonia, Dopa-responsive, Due To Sepiapterin Reductase Deficiency |
NCBI Summary: | This gene encodes an aldo-keto reductase that catalyzes the NADPH-dependent reduction of pteridine derivatives and is important in the biosynthesis of tetrahydrobiopterin (BH4). Mutations in this gene result in DOPA-responsive dystonia due to sepiaterin reductase deficiency. A pseudogene has been identified on chromosome 1. [provided by RefSeq, Jul 2008] |
UniProt Code: | P35270 |
NCBI GenInfo Identifier: | 464801 |
NCBI Gene ID: | 6697 |
NCBI Accession: | P35270.1 |
UniProt Secondary Accession: | P35270,Q53GI9, Q9UBB1, A8K741, D6W5H2, |
UniProt Related Accession: | P35270 |
Molecular Weight: | 28kDa |
NCBI Full Name: | Sepiapterin reductase |
NCBI Synonym Full Names: | sepiapterin reductase (7,8-dihydrobiopterin:NADP+ oxidoreductase) |
NCBI Official Symbol: | SPR |
NCBI Official Synonym Symbols: | SDR38C1 |
NCBI Protein Information: | sepiapterin reductase |
UniProt Protein Name: | Sepiapterin reductase |
Protein Family: | Sproutin |
UniProt Gene Name: | SPR |