Alexander J. Smith

Gene therapy for retinitis pigmentosa and Leber congenital amaurosis caused by defects in AIPL1: effective rescue of mouse models of partial and complete Aipl1 deficiency using AAV2/2 and AAV2/8 vectors (2009)

Tan, Mei Hong, Smith, Alexander J., Pawlyk, Basil, Xu, Xiaoyun, Liu, Xiaoqing, Bainbridge, James B., ...

Defects in the photoreceptor-specific gene encoding aryl hydrocarbon receptor-interacting protein-like 1 (AIPL1) are clinically heterogeneous and present as Leber Congenital Amaurosis, the severest...

Gene replacement therapy in the retinal degeneration slow (rds)mouse: the effect on retinal degeneration following partial transduction of the retina (2001)

Sarra, Gian-Marco, Stephens, Clare, De Alwis, Mahesh, Bainbridge, James W.B., Smith, Alexander J., Thrasher, Adrian J., ...

The retinal degeneration slow (rds or Prph2Rd2/Rd2) mouse, a model of recessive retinitis pigmentosa, lacks a functional gene encoding peripherin 2. This membrane glycoprotein is required for the...

MRP3, an organic anion transporter able to transport anti-cancer drugs

Kool, Marcel, Van Der Linden, Marcel, De Haas, Marcel, Scheffer, George L., Smith, Alexander J., ...

The human multidrug-resistance protein (MRP) gene family contains at least six members: MRP1, encoding the multidrug-resistance protein; MRP2 or cMOAT, encoding the canalicular multispecific organic...

Calcium-dependent Clustering of Inositol 1,4,5-Trisphosphate Receptors

Wilson, Bridget S., Pfeiffer, Janet R., Smith, Alexander J., Oliver, Janet M., Oberdorf, Jon A., Wojcikiewicz, Richard J.H.

Rat basophilic leukemia (RBL-2H3) cells predominantly express the type II receptor for inositol 1,4,5-trisphosphate (InsP3), which operates as an InsP3-gated calcium channel. In these cells,...

MRP3, an organic anion transporter able to transport anti-cancer drugs

Kool, Marcel, Van Der Linden, Marcel, De Haas, Marcel, Scheffer, George L., Smith, Alexander J., ...

The human multidrug-resistance protein (MRP) gene family contains at least six members: MRP1, encoding the multidrug-resistance protein; MRP2 or cMOAT, encoding the canalicular multispecific organic...

Calcium-dependent Clustering of Inositol 1,4,5-Trisphosphate Receptors

Wilson, Bridget S., Pfeiffer, Janet R., Smith, Alexander J., Oliver, Janet M., Oberdorf, Jon A., Wojcikiewicz, Richard J.H.

Rat basophilic leukemia (RBL-2H3) cells predominantly express the type II receptor for inositol 1,4,5-trisphosphate (InsP3), which operates as an InsP3-gated calcium channel. In these cells,...

Reaction Diffusion Modeling of Calcium Dynamics with Realistic ER Geometry

Means, Shawn, Smith, Alexander J., Shepherd, Jason, Shadid, John, Fowler, John, Wojcikiewicz, Richard J. H., ...

We describe a finite-element model of mast cell calcium dynamics that incorporates the endoplasmic reticulum's complex geometry. The model is built upon a three-dimensional reconstruction of the...