1xf9

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{{STRUCTURE_1xf9| PDB=1xf9 | SCENE= }}
{{STRUCTURE_1xf9| PDB=1xf9 | SCENE= }}
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'''Structure of NBD1 from murine CFTR- F508S mutant'''
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===Structure of NBD1 from murine CFTR- F508S mutant===
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==Overview==
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Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR), an integral membrane protein, cause cystic fibrosis (CF). The most common CF-causing mutant, deletion of Phe508, fails to properly fold. To elucidate the role Phe508 plays in the folding of CFTR, missense mutations at this position were generated. Only one missense mutation had a pronounced effect on the stability and folding of the isolated domain in vitro. In contrast, many substitutions, including those of charged and bulky residues, disrupted folding of full-length CFTR in cells. Structures of two mutant nucleotide-binding domains (NBDs) reveal only local alterations of the surface near position 508. These results suggest that the peptide backbone plays a role in the proper folding of the domain, whereas the side chain plays a role in defining a surface of NBD1 that potentially interacts with other domains during the maturation of intact CFTR.
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(as it appears on PubMed at http://www.pubmed.gov), where 15619636 is the PubMed ID number.
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{{ABSTRACT_PUBMED_15619636}}
==About this Structure==
==About this Structure==
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[[Category: Cystic fibrosis]]
[[Category: Cystic fibrosis]]
[[Category: Nucleotide-binding domain]]
[[Category: Nucleotide-binding domain]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sat May 3 14:57:12 2008''
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Sun Jul 27 14:16:27 2008''

Revision as of 11:16, 27 July 2008

Image:1xf9.png

Template:STRUCTURE 1xf9

Structure of NBD1 from murine CFTR- F508S mutant

Template:ABSTRACT PUBMED 15619636

About this Structure

1XF9 is a Single protein structure of sequence from Mus musculus. Full crystallographic information is available from OCA.

Reference

Side chain and backbone contributions of Phe508 to CFTR folding., Thibodeau PH, Brautigam CA, Machius M, Thomas PJ, Nat Struct Mol Biol. 2005 Jan;12(1):10-6. Epub 2004 Dec 26. PMID:15619636

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