Anti-RSPH3 Antibody Picoband® fluoro594 Vervoeging

Radial spoke head protein 3 homolog (RSPH3), also known as radial spoke head-like protein 2 (RSHL2), is a protein that in humans is encoded by the RSPH3 gene. The protein encoded by this gene acts as a protein kinase A anchoring protein. Mutations in this gene cause primary ciliary dyskinesia; a disorder characterized by defects of the axoneme in motile cilia and sperm flagella. The homolog of this gene was first identified in the blue-green algae Chlamydomonas as encoding a radial spoke protein that formed a structural component of motile cilia and flagella. Alternate splicing results in multiple transcript variants encoding distinct isoforms.

Protein eva-1 homolog A; Protein FAM176A; Transmembrane protein 166; EVA1A; FAM176A; TMEM166; SP24

RSPH3

83861

Q86UC2

Human

No cross-reactivity with other proteins.

E.coli-derived human RSPH3 recombinant protein (Position: Q31-H473) .

Expressed in lung, kidney, liver, pancreas, placenta, but not in heart and skeletal muscle.

Flow Cytometry

Cell Biology, Proteasome / Ubiquitin, Proteolysis/Ubiquitin, Ubiquitin E3 Enzymes

Immunogen affinity purified.

Liquid

Acts as a regulator of programmed cell death, mediating both autophagy and apoptosis.

1. Jeanson, L., Copin, B., Papon, J.-F., Dastot-Le Moal, F., Duquesnoy, P., Montantin, G., Cadranel, J., Corvol, H., Coste, A., Desir, J., Souayah, A., Kott, E., and 9 others. RSPH3 mutations cause primary ciliary dyskinesia with central-complex defects and a near absence of radial spokes. Am. J. Hum. Genet. 97: 153-162, 2015. 2. Jivan, A., Earnest, S., Juang, Y.-C., Cobb, M. H. Radial spoke protein 3 is a mammalian protein kinase A-anchoring protein that binds ERK1/2. J. Biol. Chem. 284: 29437-29445, 2009. 3. Koukoulas, I., Augustine, C., Silkenbeumer, N., Gunnersen, J. M., Scott, H. S., Tan, S.-S. Genomic organisation and nervous system expression of radial spoke protein 3. Gene 336: 15-23, 2004.

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

64 kDa

6

Eva-1 homolog A, regulator of programmed cell death

Endoplasmic reticulum membrane, Single-pass membrane protein, Lysosome membrane.

Rabbit IgG

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.