Abnormal regulatory interactions of I148T-CFTR and the epithelial Na+ channel in Xenopus oocytes.

Abnormal regulatory interactions of I148T-CFTR and the epithelial Na+ channel in Xenopus oocytes. Research Abstract Details 

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Abnormal regulatory interactions of I148T-CFTR and the epithelial Na+ channel in Xenopus oocytes. Abstract Text:

Laurence Suaud,Wusheng Yan,Ronald C Rubenstein,

The mechanisms underlying regulatory interactions of the cystic fibrosis transmembrane conductance regulator (CFTR) and the epithelial Na(+) channel (ENaC) in Xenopus oocytes are controversial. CFTR's first nucleotide binding domain (NBD-1) may be important in these interactions, because mutations within NBD-1 impair these functional interactions. We hypothesized that an abnormal CFTR containing a non-NBD-1 mutation and able to transport chloride would retain regulatory interactions with murine ENaC (mENaC). We tested this hypothesis for I148T-CFTR, where the mutation is located in CFTR's first intracellular loop. I148T-CFTR has been associated with a severe CF phenotype, perhaps because of defects in its regulation of bicarbonate transport, but it transports chloride similarly to wild-type CFTR in model systems (Choi JY, Muallem D, Kiselyov K, Lee MG, Thomas PJ, Muallem S. Nature 410: 94-97, 2001). cRNAs encoding alphabetagamma-mENaC and I148T-CFTR were injected separately or together into Xenopus oocytes. mENaC and CFTR functional expression were assessed by two-electrode voltage clamp. mENaC whole oocyte expression was determined by immunoblotting, and surface expression was quantitated by surface biotinylation. Injection of I148T-CFTR cRNA alone yielded high levels of CFTR functional expression. In coinjected oocytes, mENaC functional and surface expression was not altered by activation of I148T-CFTR with forskolin/ IBMX. Furthermore, the CFTR potentiator genistein both enhanced functional expression of I148T-CFTR and restored regulation of mENaC surface expression by activated I148T-CFTR. These data suggest that the ability to transport chloride is not a critical determinant of regulation of mENaC by activated CFTR in Xenopus oocytes and provide further evidence that I148T-CFTR is dysfunctional despite maintaining the ability to transport chloride.

Abnormal regulatory interactions of I148T-CFTR and the epithelial Na+ channel in Xenopus oocytes. Publishing Authors By Initials

L Suaud,W Yan,RC Rubenstein,

For similar animals: chordata: vertebrates: amphibia: anura: pipidae: xenopus research abstracts see: animals: chordata: vertebrates: amphibia: anura: pipidae: xenopus research

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Abnormal regulatory interactions of I148T-CFTR and the epithelial Na+ channel in Xenopus oocytes. Journal Published:

PUBLICATION TYPE: Research Support, Non-U.S. Gov

Journal: American journal of physiology. Cell physiology

VOLUME: 292

Page Numbers: C603-11

Journal Abbreviation: Am. J. Physiol., Cell Physiol.

ISSN: 0363-6143

DAY: 5

MONTH: 07

YEAR: 2006

Abnormal regulatory interactions of I148T-CFTR and the epithelial Na+ channel in Xenopus oocytes. Information

Number of References:

LANGUAGE: eng

NlmUniqueID: 100901225

Abnormal regulatory interactions of I148T-CFTR and the epithelial Na+ channel in Xenopus oocytes. Keywords Mesh Terms:

KEYWORDS: Xenopus

MESH TERMS: administration & dosage

Chemical & Substance for Abstract: Abnormal regulatory interactions of I148T-CFTR and the epithelial Na+ channel in Xenopus oocytes. Information

Substance Name: Isoleucine

Registry Number: 73-32-5

Grant and Affiliation Information for Abnormal regulatory interactions of I148T-CFTR and the epithelial Na+ channel in Xenopus oocytes.

AFFILIATION: Division of Pulmonary Medicine, Children's Hospital of Philadelphia, 34th St. and Civic Center Blvd., Philadelphia, PA 19104, USA.

Country: United States

AGENCY: United States NIDDK

GRANT: DK 58046

ACRONYM: DK

MEDLINETA: Am J Physiol Cell Physiol

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