Vestibular malformation in mice lacking Na-K-2Cl cotransporter 1 and expression of Na-K-2Cl cotransporter 1 in human vestibular end organs

Jae Young Choi, Sang Ho Jung, Wan Namkung, Ju Hyoung Lee, Eun Jin Son, Joong Wook Shin, Hun Yi Park, Won Sang Lee, Hee Nam Kim

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

Conclusion. The Na-K-2Cl cotransporter-1 (NKCCl) may be essential for the maintenance and functioning of the vestibular morphology in mice and it is strongly expressed in human vestibular end organs. Objective. NKCCl is a member of the cation-coupled chloride transporter which participates in salt transport and cell volume regulation in diverse tissues. NKCCl-deficient mice exhibit deafness, and show structural alterations in the cochlea. In addition to hearing loss, NKCCl-deficient mice show a shaker-waltzer behavior, which suggests a vestibular system defect. In this study we investigated the morphology of the vestibular system of NKCCl-deficient mice and also evaluated whether NKCCl mRNA and its protein are expressed in human vestibular end organs. Material and methods. NKCCl-deficient and wild-type mice aged 4-5 weeks were sacrificed. Their heads were cut in the midsagittal plane, fixed and decalcified. For light microscopy, 5-μm sections were cut and stained with hematoxylin-eosin. Human vestibular end organs were harvested during acoustic tumor surgery via a translabyrinthine approach. Some of these end organs were used for total mRNA extraction and the remainder for immunostaining. Reverse transcriptase polymerase chain reaction and immunostaining were performed for NKCCl. Results. The scala media of the cochleae of the NKCCl-deficient mice had collapsed but the bony labyrinth appeared unaffected. However, the semicircular canals (SCCs) were much smaller than those in the wild-type mice. Furthermore, the SCCs were completely missing in some NKCCl-deficient mice. NKCCl mRNA was expressed in both the human macula and crista ampullaris, and its protein was expressed mainly in the transitional and dark cell areas of the human crista ampullaris.

Original languageEnglish
Pages (from-to)1252-1257
Number of pages6
JournalActa Oto-Laryngologica
Volume125
Issue number12
DOIs
Publication statusPublished - 2005 Dec 1

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Semicircular Ducts
Semicircular Canals
Cochlea
Messenger RNA
Cochlear Duct
Acoustic Neuroma
Deafness
Inner Ear
Hematoxylin
Eosine Yellowish-(YS)
Reverse Transcriptase Polymerase Chain Reaction
Cell Size
Hearing Loss
Cations
Chlorides
Microscopy
Proteins
Salts
Head
Maintenance

All Science Journal Classification (ASJC) codes

  • Otorhinolaryngology

Cite this

Young Choi, Jae ; Ho Jung, Sang ; Namkung, Wan ; Lee, Ju Hyoung ; Jin Son, Eun ; Wook Shin, Joong ; Park, Hun Yi ; Sang Lee, Won ; Kim, Hee Nam. / Vestibular malformation in mice lacking Na-K-2Cl cotransporter 1 and expression of Na-K-2Cl cotransporter 1 in human vestibular end organs. In: Acta Oto-Laryngologica. 2005 ; Vol. 125, No. 12. pp. 1252-1257.
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abstract = "Conclusion. The Na-K-2Cl cotransporter-1 (NKCCl) may be essential for the maintenance and functioning of the vestibular morphology in mice and it is strongly expressed in human vestibular end organs. Objective. NKCCl is a member of the cation-coupled chloride transporter which participates in salt transport and cell volume regulation in diverse tissues. NKCCl-deficient mice exhibit deafness, and show structural alterations in the cochlea. In addition to hearing loss, NKCCl-deficient mice show a shaker-waltzer behavior, which suggests a vestibular system defect. In this study we investigated the morphology of the vestibular system of NKCCl-deficient mice and also evaluated whether NKCCl mRNA and its protein are expressed in human vestibular end organs. Material and methods. NKCCl-deficient and wild-type mice aged 4-5 weeks were sacrificed. Their heads were cut in the midsagittal plane, fixed and decalcified. For light microscopy, 5-μm sections were cut and stained with hematoxylin-eosin. Human vestibular end organs were harvested during acoustic tumor surgery via a translabyrinthine approach. Some of these end organs were used for total mRNA extraction and the remainder for immunostaining. Reverse transcriptase polymerase chain reaction and immunostaining were performed for NKCCl. Results. The scala media of the cochleae of the NKCCl-deficient mice had collapsed but the bony labyrinth appeared unaffected. However, the semicircular canals (SCCs) were much smaller than those in the wild-type mice. Furthermore, the SCCs were completely missing in some NKCCl-deficient mice. NKCCl mRNA was expressed in both the human macula and crista ampullaris, and its protein was expressed mainly in the transitional and dark cell areas of the human crista ampullaris.",
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Vestibular malformation in mice lacking Na-K-2Cl cotransporter 1 and expression of Na-K-2Cl cotransporter 1 in human vestibular end organs. / Young Choi, Jae; Ho Jung, Sang; Namkung, Wan; Lee, Ju Hyoung; Jin Son, Eun; Wook Shin, Joong; Park, Hun Yi; Sang Lee, Won; Kim, Hee Nam.

In: Acta Oto-Laryngologica, Vol. 125, No. 12, 01.12.2005, p. 1252-1257.

Research output: Contribution to journalArticle

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T1 - Vestibular malformation in mice lacking Na-K-2Cl cotransporter 1 and expression of Na-K-2Cl cotransporter 1 in human vestibular end organs

AU - Young Choi, Jae

AU - Ho Jung, Sang

AU - Namkung, Wan

AU - Lee, Ju Hyoung

AU - Jin Son, Eun

AU - Wook Shin, Joong

AU - Park, Hun Yi

AU - Sang Lee, Won

AU - Kim, Hee Nam

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N2 - Conclusion. The Na-K-2Cl cotransporter-1 (NKCCl) may be essential for the maintenance and functioning of the vestibular morphology in mice and it is strongly expressed in human vestibular end organs. Objective. NKCCl is a member of the cation-coupled chloride transporter which participates in salt transport and cell volume regulation in diverse tissues. NKCCl-deficient mice exhibit deafness, and show structural alterations in the cochlea. In addition to hearing loss, NKCCl-deficient mice show a shaker-waltzer behavior, which suggests a vestibular system defect. In this study we investigated the morphology of the vestibular system of NKCCl-deficient mice and also evaluated whether NKCCl mRNA and its protein are expressed in human vestibular end organs. Material and methods. NKCCl-deficient and wild-type mice aged 4-5 weeks were sacrificed. Their heads were cut in the midsagittal plane, fixed and decalcified. For light microscopy, 5-μm sections were cut and stained with hematoxylin-eosin. Human vestibular end organs were harvested during acoustic tumor surgery via a translabyrinthine approach. Some of these end organs were used for total mRNA extraction and the remainder for immunostaining. Reverse transcriptase polymerase chain reaction and immunostaining were performed for NKCCl. Results. The scala media of the cochleae of the NKCCl-deficient mice had collapsed but the bony labyrinth appeared unaffected. However, the semicircular canals (SCCs) were much smaller than those in the wild-type mice. Furthermore, the SCCs were completely missing in some NKCCl-deficient mice. NKCCl mRNA was expressed in both the human macula and crista ampullaris, and its protein was expressed mainly in the transitional and dark cell areas of the human crista ampullaris.

AB - Conclusion. The Na-K-2Cl cotransporter-1 (NKCCl) may be essential for the maintenance and functioning of the vestibular morphology in mice and it is strongly expressed in human vestibular end organs. Objective. NKCCl is a member of the cation-coupled chloride transporter which participates in salt transport and cell volume regulation in diverse tissues. NKCCl-deficient mice exhibit deafness, and show structural alterations in the cochlea. In addition to hearing loss, NKCCl-deficient mice show a shaker-waltzer behavior, which suggests a vestibular system defect. In this study we investigated the morphology of the vestibular system of NKCCl-deficient mice and also evaluated whether NKCCl mRNA and its protein are expressed in human vestibular end organs. Material and methods. NKCCl-deficient and wild-type mice aged 4-5 weeks were sacrificed. Their heads were cut in the midsagittal plane, fixed and decalcified. For light microscopy, 5-μm sections were cut and stained with hematoxylin-eosin. Human vestibular end organs were harvested during acoustic tumor surgery via a translabyrinthine approach. Some of these end organs were used for total mRNA extraction and the remainder for immunostaining. Reverse transcriptase polymerase chain reaction and immunostaining were performed for NKCCl. Results. The scala media of the cochleae of the NKCCl-deficient mice had collapsed but the bony labyrinth appeared unaffected. However, the semicircular canals (SCCs) were much smaller than those in the wild-type mice. Furthermore, the SCCs were completely missing in some NKCCl-deficient mice. NKCCl mRNA was expressed in both the human macula and crista ampullaris, and its protein was expressed mainly in the transitional and dark cell areas of the human crista ampullaris.

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