Evidence for a critical contribution of haploinsufficiency in the complex pathogenesis of Marfan syndrome
J. Clin. Invest. Daniel P. Judge, et al. 114:172 doi:10.1172/JCI20641 [Go to this article.]

Figure 4
(A) Immunoelectron microscopy of mouse skin using an mAb that is specific for human fibrillin-1. Left panel, nontransgenic (NonTg) control, shows no staining, confirming previously described specificity of this Ab. Middle panel, Tg(WT), and right panel, Tg(mut3), both show Ab recognition of human fibrillin-1 in a pattern that suggests the presence of both murine and human proteins within the same microfibrillar bundle. Box in the lower-right corner highlights the pattern of epitope staining, seen in black. (B) Murine and human fibrillin-1 interact. Lanes 1 and 4: ³⁵S-labeled media from murine fibroblasts coincubated with unlabeled media from murine fibroblasts before and after immunoprecipitation (IP), respectively; lanes 2 and 5: identical treatment of ³⁵S-labeled media from murine fibroblasts coincubated with unlabeled media from human fibroblasts before and after IP, respectively; lanes 3 and 6: identical treatment of ³⁵S-labeled media from human fibroblasts coincubated with unlabeled media from human fibroblasts before and after IP, respectively. Results in lane 5 demonstrate interaction between ³⁵S-labeled murine fibrillin-1 and unlabeled human fibrillin-1, while results in lane 4 attest to the specificity of the mAb for human fibrillin-1. Lane 6 serves as a positive control for IP. Position of molecular weight standards is indicated on the left.