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Please take note that our content is for educational purposes only. Be sure to contact an expert if you are unsure about the software you are installing. Do not use crack, serial number, registration code, cd key, keygen or any other type of software. to A, the *Bbs6* mRNA translated into the BBS6 protein product is non-functional and the total amount of BBS6 protein present is decreased. Therefore, any cellular phenotype associated with abrogation of the BBS6 activity in A is likely to be modified by the residual expression and activity of a wild type copy of the *Bbs6* gene in A. Similarly, the phenotypes associated with the expression of a BBS6 peptide in RPE are likely to be modified by the presence of wild type copies of the *Bbs6* gene in the other cells of the RPE as well. This is evidenced by the fact that the phenotypes of RPE expressing BBS6 cDKO or BBS6-IL clones are different from A/RPE with the cDKO cells being smaller in size than the IL clones, with more cysts present and with decreased pigmentation in the cDKO clones, whereas the phenotype of the IL clones is more like that of A and appears to be similar to the A/RPE.

*(4) Similarities between RPE cells of wild type and cDKO mice. It is known that the retinal cells of cDKO mice are smaller in size than normal retinal cells (Grieshaber et al., Nature Genet., 2000), and that the structure and function of RPE cells in eyes of cDKO mice are similar to that of RPE in A. However, the differences between the RPE of wild type and A at the mRNA level in the cDKO should not be overlooked*.

We agree with reviewer’s comments and revised the discussion.

Reviewer \#2: The manuscript by Grieshaber and collaborators reports a novel retinal degeneration model with a *Bbs6* KO mouse (A/RPE) generated by the deletion of exons 18-23 of *Bbs6* in mouse (Grieshaber et al., 2003). This *Bbs6* KO mouse is required to be co-cultured with mouse retinal explant to restore eyes. The authors have used this novel model to study the function of human BBS6 in the pathogenesis

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