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SPR

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]
Full Name
Sepiapterin Reductase
Function
Catalyzes the final one or two reductions in tetra-hydrobiopterin biosynthesis to form 5,6,7,8-tetrahydrobiopterin.
Biological Process
Biological Process nitric oxide biosynthetic processManual Assertion Based On ExperimentIDA:UniProtKB
Biological Process tetrahydrobiopterin biosynthetic processManual Assertion Based On ExperimentIBA:GO_Central
Cellular Location
Cytoplasm
Involvement in disease
Dystonia, DOPA-responsive, due to sepiapterin reductase deficiency (DRDSPRD):
A form of DOPA-responsive dystonia. 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.
PTM
In vitro phosphorylation of Ser-213 by CaMK2 does not change kinetic parameters.

Anti-SPR antibodies

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Rabbit Anti-SPR Monoclonal Antibody (EPR9289) (CBMAB-1536-YC)

Datasheet SDS
Target: SPR
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: EPR9289
Application*: WB

Rabbit Anti-SPR Monoclonal Antibody (EPR9290) (CBMAB-1535-YC)

Datasheet SDS
Target: SPR
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: EPR9290
Application*: F, IC, IF, P, IP, WB

Mouse Anti-SPR Recombinant Antibody (CBXS-2009) (CBMAB-S4776-CQ)

Datasheet SDS
Target: SPR
Host: Mouse
Specificity: Human
Clone: CBXS-2009
Application*: WB, IP, IF, E

Rabbit Anti-SPR Recombinant Antibody (CBXS-1636) (CBMAB-S4485-CQ)

Datasheet SDS
Target: SPR
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: CBXS-1636
Application*: WB, IP, P, F, IF

Rabbit Anti-SPR Recombinant Antibody (CBXS-1635) (CBMAB-S4484-CQ)

Datasheet SDS
Target: SPR
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: CBXS-1635
Application*: WB

Mouse Anti-SPR Recombinant Antibody (CBXS-5060) (CBMAB-S2281-CQ)

Datasheet SDS
Target: SPR
Host: Mouse
Antibody Isotype: IgG2a, κ
Specificity: Human
Clone: CBXS-5060
Application*: E, IP, WB

Mouse Anti-SPR Recombinant Antibody (CBXS-4861) (CBMAB-S2084-CQ)

Datasheet SDS
Target: SPR
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBXS-4861
Application*: F, IF, WB

Mouse Anti-SPR Recombinant Antibody (CBXS-4848) (CBMAB-S2072-CQ)

Datasheet SDS
Target: SPR
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human, Monkey
Clone: CBXS-4848
Application*: F, IF, WB

Mouse Anti-SPR Recombinant Antibody (CBXS-4819) (CBMAB-S2044-CQ)

Datasheet SDS
Target: SPR
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBXS-4819
Application*: F, WB

Mouse Anti-SPR Recombinant Antibody (CBXS-4753) (CBMAB-S1982-CQ)

Datasheet SDS
Target: SPR
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human, Monkey
Clone: CBXS-4753
Application*: F, IF, WB

Mouse Anti-SPR Recombinant Antibody (CBXS-4453) (CBMAB-S1695-CQ)

Datasheet SDS
Target: SPR
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBXS-4453
Application*: F, P, WB

Mouse Anti-SPR Recombinant Antibody (CBXS-4118) (CBMAB-S1373-CQ)

Datasheet SDS
Target: SPR
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human, Monkey
Clone: CBXS-4118
Application*: F, IF, WB

Mouse Anti-SPR Recombinant Antibody (CBXS-5692) (CBMAB-S0562-CQ)

Datasheet SDS
Target: SPR
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human, Rat
Clone: CBXS-5692
Application*: WB, F, IH, IF, P

Mouse Anti-SPR Recombinant Antibody (4F2) (CBMAB-A8567-LY)

Datasheet SDS
Target: SPR
Host: Mouse
Antibody Isotype: IgG2a, κ
Specificity: Human
Clone: 4F2
Application*: WB, E

SPR Matched Antibody Pair (1141) (APMAB-1141LY)

Datasheet SDS
Target: SPR
Specificity: Human

SPR Matched Antibody Pair (1140) (APMAB-1140LY)

Datasheet SDS
Target: SPR
Specificity: Human
For Research Use Only. Not For Clinical Use.
(P): Predicted
* Abbreviations
IFImmunofluorescence
IHImmunohistochemistry
IPImmunoprecipitation
WBWestern Blot
EELISA
MMicroarray
CIChromatin Immunoprecipitation
FFlow Cytometry
FNFunction Assay
IDImmunodiffusion
RRadioimmunoassay
TCTissue Culture
GSGel Supershift
NNeutralization
BBlocking
AActivation
IInhibition
DDepletion
ESELISpot
DBDot Blot
MCMass Cytometry/CyTOF
CTCytotoxicity
SStimulation
AGAgonist
APApoptosis
IMImmunomicroscopy
BABioassay
CSCostimulation
EMElectron Microscopy
IEImmunoelectrophoresis
PAPeptide Array
ICImmunocytochemistry
PEPeptide ELISA
MDMeDIP
SHIn situ hybridization
IAEnzyme Immunoassay
SEsandwich ELISA
PLProximity Ligation Assay
ECELISA(Cap)
EDELISA(Det)
BIBioimaging
IOImmunoassay
LFLateral Flow Immunoassay
LALuminex Assay
CImmunohistochemistry-Frozen Sections
PImmunohistologyp-Paraffin Sections
ISIntracellular Staining for Flow Cytometry
MSElectrophoretic Mobility Shift Assay
RIRNA Binding Protein Immunoprecipitation (RIP)
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