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RMRP is a non-coding RNA essential for early murine development.

Rosenbluh J, Nijhawan D, Chen Z, Wong KK, Masutomi K, Hahn WC - PLoS ONE (2011)

Bottom Line: RMRP mutations that lead to decreased RMRP levels are found in the pleiotropic syndrome Cartilage Hair Hypoplasia.To assess the effects of deleting RMRP, we engineered a targeting vector that contains loxP sequences flanking RMRP and created hemizygous mice harboring this engineered allele (RMRP conditional).We found that insertion of this cassette suppressed RMRP expression, and we failed to obtain viable mice homozygous for the RMRP conditional allele.

View Article: PubMed Central - PubMed

Affiliation: Department of Medical Oncology, Dana-Farber Cancer Institute, Brigham and Women's Hospital and Harvard Medical School, Boston, Massachusetts, United States of America.

ABSTRACT
RMRP is a non-coding RNA that is ubiquitously expressed in both humans and mice. RMRP mutations that lead to decreased RMRP levels are found in the pleiotropic syndrome Cartilage Hair Hypoplasia. To assess the effects of deleting RMRP, we engineered a targeting vector that contains loxP sequences flanking RMRP and created hemizygous mice harboring this engineered allele (RMRP conditional). We found that insertion of this cassette suppressed RMRP expression, and we failed to obtain viable mice homozygous for the RMRP conditional allele. Furthermore, we were unable to obtain viable homozygous RMRP mice, indicating that RMRP is essential for early embryonic development.

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The effect of RMRP depletion on neighboring genes.A. Map of the genomic structure and genes surrounding RMRP. B. Expression of RMRP, Sit1, Ccdc107, E130 and Car9 in E13.5 MEFs from the indicated genotypes. Error bars represent SD of three replicas.
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pone-0026270-g003: The effect of RMRP depletion on neighboring genes.A. Map of the genomic structure and genes surrounding RMRP. B. Expression of RMRP, Sit1, Ccdc107, E130 and Car9 in E13.5 MEFs from the indicated genotypes. Error bars represent SD of three replicas.

Mentions: The RMRP gene is located in close proximity to several other genes including Sit1, Ccdc107, E130 and Car9 (Figure 3A). To assess whether the expression of these genes are affected by RMRP depletion, we measured the expression of these genes in E13.5 MEFs derived from WT, RMRP+/− or RC+/− mice. We confirmed that RMRP levels were decreased by 50% in both RMRP+/− and RC+/− MEFs. We also found that the expression of Ccdc107 and E130 were also decreased in RMRP+/− and RC+/− MEFs while the expression of Sit1 and Car9 was not affected (Figure 3B). Although the targeting of RMRP involved the insertion of a small number of nucleotides, RMRP, Ccdc107 and E130 are located closely together, and deletion of RMRP affects the expression of all of these genes.


RMRP is a non-coding RNA essential for early murine development.

Rosenbluh J, Nijhawan D, Chen Z, Wong KK, Masutomi K, Hahn WC - PLoS ONE (2011)

The effect of RMRP depletion on neighboring genes.A. Map of the genomic structure and genes surrounding RMRP. B. Expression of RMRP, Sit1, Ccdc107, E130 and Car9 in E13.5 MEFs from the indicated genotypes. Error bars represent SD of three replicas.
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC3198473&req=5

pone-0026270-g003: The effect of RMRP depletion on neighboring genes.A. Map of the genomic structure and genes surrounding RMRP. B. Expression of RMRP, Sit1, Ccdc107, E130 and Car9 in E13.5 MEFs from the indicated genotypes. Error bars represent SD of three replicas.
Mentions: The RMRP gene is located in close proximity to several other genes including Sit1, Ccdc107, E130 and Car9 (Figure 3A). To assess whether the expression of these genes are affected by RMRP depletion, we measured the expression of these genes in E13.5 MEFs derived from WT, RMRP+/− or RC+/− mice. We confirmed that RMRP levels were decreased by 50% in both RMRP+/− and RC+/− MEFs. We also found that the expression of Ccdc107 and E130 were also decreased in RMRP+/− and RC+/− MEFs while the expression of Sit1 and Car9 was not affected (Figure 3B). Although the targeting of RMRP involved the insertion of a small number of nucleotides, RMRP, Ccdc107 and E130 are located closely together, and deletion of RMRP affects the expression of all of these genes.

Bottom Line: RMRP mutations that lead to decreased RMRP levels are found in the pleiotropic syndrome Cartilage Hair Hypoplasia.To assess the effects of deleting RMRP, we engineered a targeting vector that contains loxP sequences flanking RMRP and created hemizygous mice harboring this engineered allele (RMRP conditional).We found that insertion of this cassette suppressed RMRP expression, and we failed to obtain viable mice homozygous for the RMRP conditional allele.

View Article: PubMed Central - PubMed

Affiliation: Department of Medical Oncology, Dana-Farber Cancer Institute, Brigham and Women's Hospital and Harvard Medical School, Boston, Massachusetts, United States of America.

ABSTRACT
RMRP is a non-coding RNA that is ubiquitously expressed in both humans and mice. RMRP mutations that lead to decreased RMRP levels are found in the pleiotropic syndrome Cartilage Hair Hypoplasia. To assess the effects of deleting RMRP, we engineered a targeting vector that contains loxP sequences flanking RMRP and created hemizygous mice harboring this engineered allele (RMRP conditional). We found that insertion of this cassette suppressed RMRP expression, and we failed to obtain viable mice homozygous for the RMRP conditional allele. Furthermore, we were unable to obtain viable homozygous RMRP mice, indicating that RMRP is essential for early embryonic development.

Show MeSH
Related in: MedlinePlus