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Literature summary for 5.6.1.6 extracted from

  • Sun, F.; Mi, Z.; Condliffe, S.B.; Bertrand, C.A.; Gong, X.; Lu, X.; Zhang, R.; Latoche, J.D.; Pilewski, J.M.; Robbins, P.D.; Frizzell, R.A.
    Chaperone displacement from mutant cystic fibrosis transmembrane conductance regulator restores its function in human airway epithelia (2008), FASEB J., 22, 3255-3263.
    View publication on PubMedView publication on EuropePMC
Search for more literature for 5.6.1.6

Cloned(Commentary)

Cloned (Comment) Organism
expression of wild-type and mutant enzymes in CHO cells Homo sapiens

Protein Variants

Protein Variants Comment Organism
additional information the most common naturally occurring mutation, DELTAF508, omits the phenylalanine residue at position 508 in the first nucleotide binding domain, NBD1, of CFTR. The mutant protein is retained in the endoplasmic reticulum and degraded by the ubiquitin-proteasome system. Expression of NBD1 plus the regulatory domain of DELTAF508 CFTR, i.e. DELTAFRD, restores the biogenesis of mature DELTAF508 CFTR protein. In addition, DELTAFRD elicits a cAMP-stimulated anion conductance response in primary human bronchial epithelial cells isolated from homozygous DELTAF508 cystic fibrosis patients Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
membrane
-
 Homo sapiens 16020
-

Metals/Ions

Metals/Ions Comment Organism Structure
Mg2+ dependent on Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
additional information Homo sapiens the enzyme performs Cl- conductance. Chaperones Hsp70 and Hsp90 associate equally with wild-type and mutant DELTAF508 CFTR, whereas nearly twice as much of the Hsp90 cochaperone, Aha1, associates with mutant DELTAF508 CFTR ?
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Purification (Commentary)

Purification (Comment) Organism
wild-type and mutant enzymes partially by subcellular fractionation Homo sapiens

Source Tissue

Source Tissue Comment Organism Textmining
epithelium airway Homo sapiens
-
lung
-
 Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
additional information the enzyme performs Cl- conductance. Chaperones Hsp70 and Hsp90 associate equally with wild-type and mutant DELTAF508 CFTR, whereas nearly twice as much of the Hsp90 cochaperone, Aha1, associates with mutant DELTAF508 CFTR Homo sapiens ?
-
 ?
additional information the enzyme performs Cl- conductance Homo sapiens ?
-
 ?

Synonyms

Synonyms Comment Organism
CFTR
-
 Homo sapiens
cystic fibrosis transmembrane conductance regulator
-
 Homo sapiens

Temperature Optimum [°C]

Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
37
-
 Cl- ion transport assay at Homo sapiens