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1.
Figure 6.

Figure 6. From: Engineered telomere degradation models dyskeratosis congenita.

Telomere length manipulation in the mouse and comparison with DC Comparison of the symptoms found in DC patients with the phenotypes observed in the late generation of telomerase knockout mice and POT1b−/− and POT1b−/−mTR+/− mice.

Dirk Hockemeyer, et al. Genes Dev. 2008 July 1;22(13):1773-1785.
2.
Figure 3.

Figure 3. From: Engineered telomere degradation models dyskeratosis congenita.

Cutanous phenotypes. (A) Photographs of POT1bS/S and age-matched control animals showing hyperpigmentation on the snout, ears, tail, and paws of POT1bS/S animals. (B) Hyperpigmentation of the paws of mice with the indicated genotypes and ages. (C) H&E and Fontana-Masson staining of tail sections of 8-mo-old mice with the indicated genotypes. Arrows indicate the increased deposition of melanosomes over the nuclei of the hyperpigmented mouse tail sections. Arrowheads indicate retention of the melanin in the stratum corneum. Enlarged images of the Fontana-Masson staining of tail sections of POT1b−/− and wild-type mice are shown at right. (D) Photographs showing abnormal nails on one paw of an 11-mo-old G5 POT1S/S mouse. (Top) The wild-type control is age-matched.

Dirk Hockemeyer, et al. Genes Dev. 2008 July 1;22(13):1773-1785.
3.
Figure 5.

Figure 5. From: Engineered telomere degradation models dyskeratosis congenita.

Telomere dysfunction in bone marrow of POT1b−/−mTR+/− mice. (A) Q-FISH analysis of the metaphases isolated from bone marrow of 3-mo-old littermates with the indicated genotypes. The X axis shows telomere fluorescence hybridization intensity (TFU), the Y axis shows the frequency of chromosomes ends with the indicated TFUs. Average TFU values are indicated in each panel. (B) Telomere FISH analysis of metaphases isolated from the bone marrow of mice (3-mo-old littermates) with the indicated genotypes. DAPI stained chromosomes are false colored in red and the telomere hybridization signal is shown in green. (C) Quantification of chromosome abnormalities detected in metaphases as shown in B. Controls represent pooled data obtained from bone marrow samples of two POT1b+/− mTR+/−, two POT1b+/−mTR−/−, and two POT1b−/− mTR+/+ mice. tel+ and tel indicate fusions with and without telomeric FISH signals at the fusions sites, respectively.

Dirk Hockemeyer, et al. Genes Dev. 2008 July 1;22(13):1773-1785.
4.
Figure 1.

Figure 1. From: Engineered telomere degradation models dyskeratosis congenita.

Accelerated telomere shortening in POT1b-deficient cells. (A) Telomere length dynamics and telomeric overhangs of SV40LT-immortalized mTR-proficient and -deficient MEFs before and after deletion of POT1b with Cre. (Top) Size-fractionated MboI-digested DNA hybridized in gel under native conditions with a radiolabeled [CCCTAA]4-oligo detecting the 3′ overhang. (Bottom) The same gels after in situ denaturation of the DNA and rehybridization with the same probe. Numbers above the lanes reflect population doublings after introduction of pWZL-hygro-Cre (or the empty vector). MWs are indicated in kilobases on the left. (B) Telomere FISH analysis on metaphase chromosome spreads of the cells analyzed in A. PDs are given in C. DAPI-stained chromosomes are false colored the in red, and the telomere hybridization signal is shown in green. Arrows point at chromosome fusions. (C) Quantification of chromosome fusions detected in metaphases as shown in B.

Dirk Hockemeyer, et al. Genes Dev. 2008 July 1;22(13):1773-1785.
5.
Figure 2.

Figure 2. From: Engineered telomere degradation models dyskeratosis congenita.

Curtailed telomerase exacerbates POT1b KO phenotypes. (A) Genotypes of 281 offspring from intercrosses between 15 POT1b+/−mTR+/− breeding pairs. (B) Photograph showing the single POT1b−/−mTR−/− animal born and its littermates. (C) Scatter plot of the bodyweights of adult (>3 mo) female and male mice with the indicated genotypes. All animals were age-matched and derived from the intercross described in A. Error bars indicate the standard error of the mean. (D) Quantification of testis size of age-matched males of the indicated genotypes. Error bars indicate the standard error of the mean. (E) H&E stained testis sections at two magnifications. Mice as in A. (F) Incidence of testicular atrophy in mice of the indicated genotypes. Grading is based on H&E staining as shown in E. (G) TUNEL assay on H&E-stained sections from the small intestine of age-matched mice of the indicated genotypes. (H) Quantification of apoptotic cells in the small intestines based on TUNEL staining of age-matched mice with the indicated genotypes generated as in A. (POT1b+/+mTR+/+, POT1b+/+mTR+/−, POT1b+/−mTR+/− n = 4; POT1b−/−mTR+/+ and POT1b−/−mTR+/− n = 5). For each animal >60 crypts were analyzed. Error bars indicate the standard error of the mean.

Dirk Hockemeyer, et al. Genes Dev. 2008 July 1;22(13):1773-1785.
6.
Figure 4.

Figure 4. From: Engineered telomere degradation models dyskeratosis congenita.

Bone marrow failure and premature death in POT1b−/−mTR+/− mice. (A) Peripheral blood counts of age-matched mice with the indicated genotypes (POT1b+/+mTR+/+, n = 6, average age: 149 d; POT1b+/+mTR+/−, n = 5, average age: 132 d; POT1b+/−mTR+/−, n = 6, average age: 137 d; POT1b−/−mTR+/+, n = 9, average age: 138 d; POT1b−/−mTR+/−, n = 8, average age: 110 d). Error bars indicate the standard error of the mean. Differences between POT1b−/−mTR+/+ animals and POT1b−/−mTR+/− mice are significant (P < 0.05, upaired t-test) for all parameters except for the red blood cells counts. (B) H&E staining of bone marrow section of a 121-d-old POT1b+/−mTR+/− mouse (left panels) and an 83-d-old POT1b−/−mTR+/− mouse (right panels). (C) Immunohistochemical staining of B220 and Ter119 in spleen sections of mice with the indicated genotypes. (age: POT1b+/+mTR+/+ and POT1b−/−mTR+/+ animals 179 d, POT1+/−mTR+/− and POT1b−/−mTR+/− animals 121 d). (D) Kaplan-Meyer survival plot of mice derived from a heterozygous intercross of POT1b+/−mTR+/− mice (see Fig. 2A). POT1b−/−mTR+/− mice die prematurely compared with the controls (P < 0.0001, based on Log rank test).

Dirk Hockemeyer, et al. Genes Dev. 2008 July 1;22(13):1773-1785.

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