|Positive WB detected in||mouse eye tissue|
|Positive IF detected in||mouse Retina tissue|
|Western Blot (WB)||WB : 1:500-1:2000|
|Immunofluorescence (IF)||IF : 1:20-1:200|
|Sample-dependent, check data in validation data gallery|
The immunogen of 18109-1-AP is PRPH2 Fusion Protein expressed in E. coli.
|Tested Reactivity||human, mouse, rat|
|Cited Reactivity||human, mouse, rat|
|Host / Isotype||Rabbit / IgG|
|Immunogen||PRPH2 fusion protein Ag12555|
|Full Name||peripherin 2 (retinal degeneration, slow)|
|Calculated molecular weight||346 aa, 39 kDa|
|Observed molecular weight||39 kDa|
|GenBank accession number||BC074720|
|Gene ID (NCBI)||5961|
|Purification Method||Antigen affinity purification|
|Storage Buffer||PBS with 0.02% sodium azide and 50% glycerol pH 7.3.|
|Storage Conditions||Store at -20°C. Stable for one year after shipment. Aliquoting is unnecessary for -20oC storage.|
Peripherin-2 (PRPH2), also known as retinal degeneration slow protein (RDS), is a photoreceptor-specific tetraspanin protein implicated in outer segment disk morphogenesis. It may function as an adhesion molecule involved in stabilization and compaction of outer segment disks or in the maintenance of the curvature of the rim. Mutations in peripherin-2 are responsible for various retinal degenerative diseases including autosomal dominant retinitis pigmentosa (ADRP).
The Late Endosomal Pathway Regulates the Ciliary Targeting of Tetraspanin Protein Peripherin 2.
Multimeric conformation of type III intermediate filaments but not the filamentous conformation exhibits high affinity to lipid bilayers.
Differential requirement of NPHP1 for compartmentalized protein localization during photoreceptor outer segment development and maintenance.
J Biol Chem
The myosin-tail homology domain of centrosomal protein 290 is essential for protein confinement between the inner and outer segments in photoreceptors.
Evaluation of micro Electroretinograms Recorded with Multiple Electrode Array to Assess Focal Retinal Function.
Hum Mol Genet
BBS mutations modify phenotypic expression of CEP290-related ciliopathies.