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Cleft Palate, Moderate Lung Developmental Retardation and Early Postnatal Lethality in Mice Deficient in the Kir7.1 Inwardly Rectifying K+ Channel.

Villanueva S, Burgos J, López-Cayuqueo KI, Lai KM, Valenzuela DM, Cid LP, Sepúlveda FV - PLoS ONE (2015)

Bottom Line: Kir7.1 is present in epithelial tissues where it colocalizes with the Na+/K+-pump probably serving to recycle K+ taken up by the pump.Kir7.1 is expressed in the epithelium covering the palatal processes at the time at which palate sealing takes place and our results suggest it might play an essential role in late palatogenesis.Our work also reveals a second unexpected role in the development and the physiology of the respiratory system, where Kir7.1 is expressed in epithelial cells all along the respiratory tree.

View Article: PubMed Central - PubMed

Affiliation: Centro de Estudios Científicos (CECs), Valdivia, Chile.

ABSTRACT
Kir7.1 is an inwardly rectifying K+ channel of the Kir superfamily encoded by the kcnj13 gene. Kir7.1 is present in epithelial tissues where it colocalizes with the Na+/K+-pump probably serving to recycle K+ taken up by the pump. Human mutations affecting Kir7.1 are associated with retinal degeneration diseases. We generated a mouse lacking Kir7.1 by ablation of the Kcnj13 gene. Homozygous mutant mice die hours after birth and show cleft palate and moderate retardation in lung development. Kir7.1 is expressed in the epithelium covering the palatal processes at the time at which palate sealing takes place and our results suggest it might play an essential role in late palatogenesis. Our work also reveals a second unexpected role in the development and the physiology of the respiratory system, where Kir7.1 is expressed in epithelial cells all along the respiratory tree.

No MeSH data available.


Related in: MedlinePlus

Palate formation during embryonic development in Kcnj13+/+, Kcnj13+/- and Kcnj13-/- mice.a. Dissected midfacial segments (without brain, mandible and tongue) from mouse heads collected from embryos 13.5–16.5 dpc. b. Mouse heads on postnatal day (P0) with dissected midfacial segments (left) and alizarin red and alcian blue skeletal staining (right). The palatine (pp) and maxillary (mp) processes are indicated In the Kcnj13-/- specimen both the presphenoid (ps) and the vomer (v) are visible. Bar, 1 mm.
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pone.0139284.g005: Palate formation during embryonic development in Kcnj13+/+, Kcnj13+/- and Kcnj13-/- mice.a. Dissected midfacial segments (without brain, mandible and tongue) from mouse heads collected from embryos 13.5–16.5 dpc. b. Mouse heads on postnatal day (P0) with dissected midfacial segments (left) and alizarin red and alcian blue skeletal staining (right). The palatine (pp) and maxillary (mp) processes are indicated In the Kcnj13-/- specimen both the presphenoid (ps) and the vomer (v) are visible. Bar, 1 mm.

Mentions: Another cause of early postnatal lethality in mouse models is a developmental craniofacial alteration leading to cleft palate [20] and a recent study of mouse palatal transcriptome suggests Kcnj13 gene as a potential key regulator of palatal development [21]. Kcjn13-/- mice do indeed show a deficiency in the process of palate fusion during development. Palate formation in mice takes place by fusion of so called palatal shelves that are paired outgrowths initially growing vertically flanking the developing tongue (~13.5 dpc) to then progressing horizontally towards the midline above the tongue (~14.5 dpc). Fusion of the palatal processes has already occurred by dpc 15.5 [22]. Fig 5a shows macroscopic views of the roof of the mouth of Kcjn13+/+, Kcjn13+/- and Kcjn13-/- mice. Palatal processes that are evident at 13.5 and 14.5 dpc in WT and heterozygous embryos have disappeared at 15.5 dpc, at which time complete sealing of the palate is evident. This was not the case for Kcjn13-/- embryos that show a delayed horizontal growth from E14.5 and lack of fusion at 15.5 and 16.5 dpc. Fig 5b shows the status of palate fusion in Kcjn13+/+, Kcjn13+/- and Kcjn13-/- newborn mice, with the latter presenting evident cleft palate. Also shown are preparations to reveal bone and cartilage (blue), where it can be seen that palatine (pp) and maxillary processes (mp) are extended to the midline in Kcjn13+/+ and Kcjn13+/- tissues. In the Kcjn13-/- mouse, these processes were absent thus exposing the vomer (v) and presphenoid (ps) bones to result in a complete cleft secondary palate.


Cleft Palate, Moderate Lung Developmental Retardation and Early Postnatal Lethality in Mice Deficient in the Kir7.1 Inwardly Rectifying K+ Channel.

Villanueva S, Burgos J, López-Cayuqueo KI, Lai KM, Valenzuela DM, Cid LP, Sepúlveda FV - PLoS ONE (2015)

Palate formation during embryonic development in Kcnj13+/+, Kcnj13+/- and Kcnj13-/- mice.a. Dissected midfacial segments (without brain, mandible and tongue) from mouse heads collected from embryos 13.5–16.5 dpc. b. Mouse heads on postnatal day (P0) with dissected midfacial segments (left) and alizarin red and alcian blue skeletal staining (right). The palatine (pp) and maxillary (mp) processes are indicated In the Kcnj13-/- specimen both the presphenoid (ps) and the vomer (v) are visible. Bar, 1 mm.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4581704&req=5

pone.0139284.g005: Palate formation during embryonic development in Kcnj13+/+, Kcnj13+/- and Kcnj13-/- mice.a. Dissected midfacial segments (without brain, mandible and tongue) from mouse heads collected from embryos 13.5–16.5 dpc. b. Mouse heads on postnatal day (P0) with dissected midfacial segments (left) and alizarin red and alcian blue skeletal staining (right). The palatine (pp) and maxillary (mp) processes are indicated In the Kcnj13-/- specimen both the presphenoid (ps) and the vomer (v) are visible. Bar, 1 mm.
Mentions: Another cause of early postnatal lethality in mouse models is a developmental craniofacial alteration leading to cleft palate [20] and a recent study of mouse palatal transcriptome suggests Kcnj13 gene as a potential key regulator of palatal development [21]. Kcjn13-/- mice do indeed show a deficiency in the process of palate fusion during development. Palate formation in mice takes place by fusion of so called palatal shelves that are paired outgrowths initially growing vertically flanking the developing tongue (~13.5 dpc) to then progressing horizontally towards the midline above the tongue (~14.5 dpc). Fusion of the palatal processes has already occurred by dpc 15.5 [22]. Fig 5a shows macroscopic views of the roof of the mouth of Kcjn13+/+, Kcjn13+/- and Kcjn13-/- mice. Palatal processes that are evident at 13.5 and 14.5 dpc in WT and heterozygous embryos have disappeared at 15.5 dpc, at which time complete sealing of the palate is evident. This was not the case for Kcjn13-/- embryos that show a delayed horizontal growth from E14.5 and lack of fusion at 15.5 and 16.5 dpc. Fig 5b shows the status of palate fusion in Kcjn13+/+, Kcjn13+/- and Kcjn13-/- newborn mice, with the latter presenting evident cleft palate. Also shown are preparations to reveal bone and cartilage (blue), where it can be seen that palatine (pp) and maxillary processes (mp) are extended to the midline in Kcjn13+/+ and Kcjn13+/- tissues. In the Kcjn13-/- mouse, these processes were absent thus exposing the vomer (v) and presphenoid (ps) bones to result in a complete cleft secondary palate.

Bottom Line: Kir7.1 is present in epithelial tissues where it colocalizes with the Na+/K+-pump probably serving to recycle K+ taken up by the pump.Kir7.1 is expressed in the epithelium covering the palatal processes at the time at which palate sealing takes place and our results suggest it might play an essential role in late palatogenesis.Our work also reveals a second unexpected role in the development and the physiology of the respiratory system, where Kir7.1 is expressed in epithelial cells all along the respiratory tree.

View Article: PubMed Central - PubMed

Affiliation: Centro de Estudios Científicos (CECs), Valdivia, Chile.

ABSTRACT
Kir7.1 is an inwardly rectifying K+ channel of the Kir superfamily encoded by the kcnj13 gene. Kir7.1 is present in epithelial tissues where it colocalizes with the Na+/K+-pump probably serving to recycle K+ taken up by the pump. Human mutations affecting Kir7.1 are associated with retinal degeneration diseases. We generated a mouse lacking Kir7.1 by ablation of the Kcnj13 gene. Homozygous mutant mice die hours after birth and show cleft palate and moderate retardation in lung development. Kir7.1 is expressed in the epithelium covering the palatal processes at the time at which palate sealing takes place and our results suggest it might play an essential role in late palatogenesis. Our work also reveals a second unexpected role in the development and the physiology of the respiratory system, where Kir7.1 is expressed in epithelial cells all along the respiratory tree.

No MeSH data available.


Related in: MedlinePlus