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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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RMRP depletion leads to reduced levels of RMRP transcript.Total RNA was produced from E13.5 MEFs and RMRP level was measured by A. qRT-PCR B. Northern blot using either a sense or antisense RMRP probe. Error bars represent SD of three replicas.
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pone-0026270-g002: RMRP depletion leads to reduced levels of RMRP transcript.Total RNA was produced from E13.5 MEFs and RMRP level was measured by A. qRT-PCR B. Northern blot using either a sense or antisense RMRP probe. Error bars represent SD of three replicas.

Mentions: The levels of RMRP may be critical for RMRP function. Specifically, Nakashima et al. have proposed a model by which RMRP mutations found in CHH patients leads to destabilization of RMRP [3]. When we assessed total levels of RMRP in murine embryonic fibroblasts (MEFs) obtained from RMRP or RC hemizygous mice, we found that RMRP was expressed at 50% of the level found in wild type MEFs (Figure 2A). RC and RMRP+/− mice were monitored from birth to 18 months of age and no abnormality in size, fur or behavior was detected. This is in consonance with what has been observed in human carriers of RMRP mutations [2].


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)

RMRP depletion leads to reduced levels of RMRP transcript.Total RNA was produced from E13.5 MEFs and RMRP level was measured by A. qRT-PCR B. Northern blot using either a sense or antisense RMRP probe. Error bars represent SD of three replicas.
© Copyright Policy
Related In: Results  -  Collection

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

pone-0026270-g002: RMRP depletion leads to reduced levels of RMRP transcript.Total RNA was produced from E13.5 MEFs and RMRP level was measured by A. qRT-PCR B. Northern blot using either a sense or antisense RMRP probe. Error bars represent SD of three replicas.
Mentions: The levels of RMRP may be critical for RMRP function. Specifically, Nakashima et al. have proposed a model by which RMRP mutations found in CHH patients leads to destabilization of RMRP [3]. When we assessed total levels of RMRP in murine embryonic fibroblasts (MEFs) obtained from RMRP or RC hemizygous mice, we found that RMRP was expressed at 50% of the level found in wild type MEFs (Figure 2A). RC and RMRP+/− mice were monitored from birth to 18 months of age and no abnormality in size, fur or behavior was detected. This is in consonance with what has been observed in human carriers of RMRP mutations [2].

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