Anti-SPR Antibody (CAB19859)[KO Validated]

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SKU:
CAB19859
Product type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Research Area:
Cell Biology
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Description

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抗体名:Anti-SPR Antibody [KO Validated]
抗体コード:CAB19859
抗体サイズ:20uL, 50uL, 100uL
申し込み:WB
反応性:Human, Mouse, Rat
宿主種:Rabbit
免疫原:Recombinant protein of human SPR.
申し込み:WB
推奨希釈:WB 1:500 - 1:2000
反応性:Human, Mouse, Rat
ポジティブサンプル:HeLa
免疫原:Recombinant protein of human SPR.
精製方法:Affinity purification
ストレージバッファ:Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
アイソタイプ:IgG
順序:Email for sequence
遺伝子ID:6697
Uniprot:P35270
セルラーロケーション:Cytoplasm
計算された分子量:28kDa
観察された分子量:28KDa
Anti-SPR Antibody [KO Validated] (CAB19859)Western blot analysis of extracts from normal (control) and SPR knockout (KO) HeLa cells, using SPR antibody (CAB19859) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 1s.
同義語:SPR, SDR38C1
バックグラウンド: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.
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  
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