Independent degeneration of photoreceptors and retinal pigment epithelium in conditional knockout mouse models of choroideremia
J. Clin. Invest. Tanya Tolmachova, et al. 116:386 doi:10.1172/JCI26617 [
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Figure 2Analysis of Rep1 protein amount and function. (
A) Total protein lysates from stomach of
gunmetal (
gm/gm),
Chmnull/WT, and
ChmWT/WT mice were subjected to subcellular fractionation, and total (T), soluble (S), and pellet (P) fractions were immunoblotted and probed with anti-Rab27a antibody 4B12, anti-Rab8 antibody, and anti-Rab11 antibody as indicated. Calnexin was used as a control for subcellular fractionation. (
B) Immunoblot of total protein lysates from lung, liver, and kidney of
Chm3lox/Y,
Chmflox/Y, and
ChmWT/Y mice probed with anti-Rep antibody J905, which recognizes both Rep1 and Rep2 proteins. (
C) Total protein lysates from large intestines of
Chm3lox/Y,
Chmflox/Y, and
ChmWT/Y mice were subjected to subcellular fractionation, and total (T), soluble (S), and pellet (P) fractions were immunoblotted and probed with anti-Rab27a antibody 4B12. Calnexin was used as a control for subcellular fractionation. (
D and
E) In vitro prenylation was carried out on the soluble fraction of total protein lysates from large intestines (
D) and eyes (
E). The
Chm alleles are indicated above the lanes. “
MCM” indicates mice carrying the
MerCreMer transgene. “+TM” indicates treatment with TM. The radiogram underneath shows a shorter exposure to film.