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CF, like DPB, causes severe lung inflammation, abundant mucus production, infection, and shows a genetic predominance among Caucasians of one geographic group to the rarity of others; whereas DPB dominates among East Asians, CF mainly affects individuals of European descent.
This may explain why mutations or single nucleotide polymorphisms (SNPs) of these receptors have the modifier function in CF or CF like diseases[37] [39].
More specifically, the levels of LXA4 have been reported to be decreased in CF, like in other chronic airway inflammatory diseases such as asthma [ 29, 52– 52].
It has recently been introduced to UK and Europe and has been used in several other pulmonary diseases, different from CF like chronic obstructive pulmonary disease [ 22] or exacerbations of chronic obstructive pulmonary disease or bronchial asthma [ 23].
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Taken together, ΔF-CFTR inhibition of WT-CFTR during protein processing in the ER of native CF human bronchial epithelial cells explains CF-like disease symptoms but not fully developed CF disease in CF heterozygous carriers, the majority of whom are WT/ΔF carriers in the overall population.
The βENaC-transgenic mouse phenocopies increased airway Na+ absorption and airway surface liquid depletion characteristic of human CF airways and develops spontaneous CF-like lung disease with mucus plugging, chronic airway inflammation and reduced clearance of bacterial pathogens [3,4].
In this model, airway-specific overexpression of the β-subunit of the epithelial Na+ channel ENaC mimics airway surface dehydration characteristic for CF airways and produces a CF-like lung disease with early onset of mucus obstruction, chronic airway inflammation, slowed bacterial clearance, and progressive structural lung damage [53].
The potential role of YKL-40 for the pathogenesis of CF lung disease is further supported by findings in Scnn1b-transgenic mice, a murine model of CF-like lung disease [51,52].
The Beam Gas Ionisation (BGI) instrument for the CERN Proton Synchrotron accelerator will require a CF-like rectangular sealing system.
Next, they mated these pigs to see if offspring with two bad copies of CFTR develop a CF-like disease.
This classical candidate gene approach provided several novel insights into the pathogenesis of early CF-like lung disease that may support the development of novel therapies [15].
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