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The role of the UPS in cystic fibrosis
CF is an inherited autosomal recessive disease whose lethality arises from malfunction of CFTR, a single chloride (Cl(-)) ion channel protein. CF patients harbor mutations in the CFTR gene that lead to misfolding of the resulting CFTR protein, rendering it inactive and mislocalized. Hundreds of CF-r...
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| 主要な著者: | , , |
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| フォーマット: | Artigo |
| 言語: | Inglês |
| 出版事項: |
BioMed Central
2007
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| 主題: | |
| オンライン･アクセス: | https://ncbi.nlm.nih.gov/pmc/articles/PMC2106362/ https://ncbi.nlm.nih.gov/pubmed/18047735 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/1471-2091-8-S1-S11 |
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