Product Name :
SPR Recombinant Rabbit Monoclonal Antibody [PSH06-14]
Predicted band size :
28 kDa
Observed band size :
28 kDa
Synonyms:
OTTHUMP00000160199 antibody SDR38C1 antibody Sepiapterin reductase (7,8 dihydrobiopterin:NADP+ oxidoreductase) antibody Sepiapterin reductase antibody Short chain dehydrogenase/reductase family 38C, member 1 antibody SPR antibody SPRE_HUMAN antibody
Function :
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. 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.941289-27-6 site Dystonia is defined by the presence of sustained involuntary muscle contractions, often leading to abnormal postures.4-Chloro-6-methyl-7-azaindole Chemscene
Antibody Type:
Recombinant Rabbit monoclonal Antibody
Immunogen :
Recombinant protein within Human SPR aa 1-261.
Species reactivity:
Human
Verify application:
WB, IHC-P, IF-Cell, FC
Coupling :
unconjugated
Clone number:
PSH06-14
RRID:
Form :
Liquid
Concentration :
1ug/ul
Storage instructions:
Store at +4℃ after thawing.PMID:33417700 Aliquot store at -20℃. Avoid repeated freeze / thaw cycles.
Storage buffer solution:
PBS (pH7.4), 0.1% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.
Subtype:
IgG
Purification method :
Protein A affinity purified.
ELTSA :
Molecular weight :
Predicted band size: 28 kDa
Positive control :
A549 cell lysate, MCF7 cell lysate, HeLa cell lysate, HepG2 cell lysate, 293T cell lysate, SH-SY5Y cell lysate, HeLa, human thyroid gland cancer tissue.