Product Name	PDE6B
Chinese Name	磷酸二酯酶6β抗体
Alias	5''-cyclic phosphodiesterase subunit beta; Congenital stationary night blindness 3 autosomal dominant; CSNB 3; CSNB3; CSNBAD2; GMP PDE beta; GMP-PDE beta; PDE 6 beta; PDE 6B; PDE6B; PDE6B_HUMAN; PDEB; Phosphodiesterase 6B; Phosphodiesterase 6B cGMP specific rod beta; Rd 1; Rd1; Rod cGMP phosphodiesterase beta subunit; Rod cGMP specific 3' 5' cyclic phosphodiesterase beta subunit; Rod cGMP-specific 3''; RP40.
Research Area	Cell biology  Neurobiology  Signal transduction
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	(predicted: Human, Mouse, Rat, )
Applications	WB=1:500-2000 ELISA=1:5000-10000  not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	66kDa
Cellular localization	The cell membrane
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human PDE6B: 2-100/854
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	Photon absorption triggers a signaling cascade in rod photoreceptors that activates cGMP phosphodiesterase (PDE), resulting in the rapid hydrolysis of cGMP, closure of cGMP-gated cation channels, and hyperpolarization of the cell. PDE is a peripheral membrane heterotrimeric enzyme made up of alpha, beta, and gamma subunits. This gene encodes the beta subunit. Mutations in this gene result in retinitis pigmentosa and autosomal dominant congenital stationary night blindness. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Feb 2009] Function: This protein participates in processes of transmission and amplification of the visual signal. Necessary for the formation of a functional phosphodiesterase holoenzyme. Subcellular Location: Membrane. DISEASE: Defects in PDE6B are the cause of retinitis pigmentosa type 40 (RP40) [MIM:613801]. RP40 is a retinal dystrophy belonging to the group of pigmentary retinopathies. Retinitis pigmentosa is characterized by retinal pigment deposits visible on fundus examination and primary loss of rod photoreceptor cells followed by secondary loss of cone photoreceptors. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well. Defects in PDE6B are a cause of congenital stationary night blindness autosomal dominant type 2 (CSNBAD2) [MIM:163500]; also known as congenital stationary night blindness Rambusch type. Congenital stationary night blindness is a non-progressive retinal disorder characterized by impaired night vision. Similarity: Belongs to the cyclic nucleotide phosphodiesterase family. Contains 2 GAF domains. SWISS: P35913 Gene ID: 5158 Database links: Entrez Gene: 281974 Cow Entrez Gene: 399653 Dog Entrez Gene: 5158 Human Entrez Gene: 18587 Mouse Entrez Gene: 289878 Rat Omim: 180072 Human SwissProt: P23439 Cow SwissProt: P33726 Dog SwissProt: P35913 Human SwissProt: P23440 Mouse Unigene: 623810 Human Unigene: 654544 Human Unigene: 1372 Mouse

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