About

Camille Ehre is a faculty member at the UNC School of Medicine, working within the Curriculum in Toxicology & Environmental Medicine. Her research focuses on the biochemical and biophysical properties of mucus, particularly how these properties are affected in various airway diseases such as asthma, cystic fibrosis (CF), and primary ciliary dyskinesia (PCD). Her work aims to understand the mechanisms underlying mucus dysfunction in respiratory diseases, contributing to the development of targeted therapies and interventions for airway conditions.

Research topics

• Biology
• Medicine
• Virology
• Genetics
• Internal medicine
• Immunology
• Pathology
• Biophysics
• Biochemistry
• Materials science
• Chemistry
• Gastroenterology

Selected publications

• iPSC-derived human intestinal organoids to investigate gastrointestinal pathophysiology in cystic fibrosis

  Physiology · 2026-05-01

  article

  In addition to pulmonary disease, people with cystic fibrosis (pwCF) can experience gastrointestinal (GI) complications, including impaired nutrient uptake, obstruction, and elevated risk of GI cancers. Despite the use of highly effective modulator therapies, CF-associated GI manifestations persist, and the mechanisms driving them are comparatively underexplored. Progress has been limited by the absence of models that reliably reproduce the human intestinal mucosal environment: animal models fail to capture human small intestinal physiology, and current in vitro approaches are unable to generate the full diversity of cell types. To address this gap, we developed an innovative human intestinal organoid model derived from induced pluripotent stem cells (iPSCs) to investigate the mechanisms underlying the pathophysiology of the CF intestine. We used this platform to test the hypothesis that altered mucus composition and aberrant intestinal cell biology impairs nutrient absorption in pwCF. We generated human intestinal organoids (HIOs) from iPSCs carrying common CFTR mutations (F508del, G542X, and W1282X) and compared them to wild-type controls. Upon xenograft, transplanted organoids (tHIOs) from all CFTR variants grew and matured to exhibit the distinct crypt-villus architecture of the small intestine. We used immunofluorescence staining to identify differentiated cell types, including enteroendocrine, Paneth, goblet, and BEST4 cells in tHIOs from all CFTR variants. Histological and immunofluorescence analysis revealed that CF tHIOS exhibited increased mucus retention and a shift toward acidic mucins, with a pronounced retention of MUC2 in goblet cells. Additionally, wheat germ agglutinin staining demonstrated notable glycoprotein accumulation clogging the crypts and lining the epithelium of CF tHIOs. We collected secreted mucus from the enclosed luminal space of mature tHIOs which revealed an elevated percent solid composition in CF tHIOs compared to controls. We are currently using Ussing chambers, enteroid cultures, and in vivo exposure to nutrients to define how this abnormal mucus may alter the absorptive capacity of CF small intestinal epithelium. This model provides a robust platform for modeling CF-associated GI pathophysiology, offering new opportunities to study manifestations of the disease. This human system is advantageous because it allows direct examination of aberrant CF small intestinal mucus without interference from the microbiome and other luminal factors. Additionally, tHIOs provide an opportunity to define how CFTR dysfunction disrupts intestine development, stem cell dynamics, and nutrient absorption, ultimately guiding the design of therapeutic strategies to mitigate GI complications in pwCF. This work was supported by the Cystic Fibrosis Foundation (MCCAUL23G0-GI) and a Pilot and Feasibility award from the UNC CFRTCC. This abstract was presented at the American Physiology Summit 2026 and is only available in HTML format. There is no downloadable file or PDF version. The Physiology editorial board was not involved in the peer review process.

  PublisherDOI

• Mucus and inflammation in the HEMT Era: Persistent barriers to complete airway normalization in cystic fibrosis

  Journal of Cystic Fibrosis · 2026-03-01 · 2 citations

  articleOpen access1st authorCorresponding
  PublisherOA PDFDOI

• Differential responses to e-cig generated aerosols from humectants and different forms of nicotine in epithelial cells from nonsmokers and smokers

  UNC Libraries · 2026-02-12

  articleOpen access1st authorCorresponding

  In the United States, millions of adults use electronic cigarettes (e-cigs), and a majority of these users are former or current cigarette smokers. It is unclear, whether prior smoking status affects biological responses induced by e-cigs. In this study, differentiated human nasal epithelial cells (hNECs) from nonsmokers and smokers at air-liquid interface were acutely exposed to the e-cig generated aerosols of humectants, propylene glycol (PG), and glycerol (GLY). Mucin levels were examined in the apical washes, and cytokine levels were assessed in the basolateral supernatants 24 h postexposure. The aerosol from the GLY exposure increased mucin 5, subtype AC (MUC5AC) levels in the apical wash of hNECs from nonsmokers, but not smokers. However, the aerosol from GLY induced pro-inflammatory responses in hNECs from smokers. We also exposed hNECs from nonsmokers and smokers to e-cig generated aerosol from PG:GLY with freebase nicotine or nicotine salt. The PG:GLY with freebase nicotine exposure increased MUC5AC and mucin 5, subtype B (MUC5B) levels in hNECs from nonsmokers, but the nicotine salt exposure did not. The PG:GLY with nicotine salt exposure increased pro-inflammatory cytokines in hNECs from smokers, which was not seen with the freebase nicotine exposure. Taken together, these data indicate that the e-cig generated aerosols from the humectants, mostly GLY, and the type of nicotine used cause differential effects in airway epithelial cells from nonsmokers and smokers. As e-cig use is increasing, it is important to understand that the biological effects of e-cig use are likely dependent on prior cigarette smoke exposure.

  PublisherDOI

• Airway epithelial SARS-CoV-2 infectious and repair responses: relationships to age, sex, and post-COVID pulmonary syndromes

  bioRxiv (Cold Spring Harbor Laboratory) · 2025-07-17

  preprintOpen access

  Abstract The long-term pulmonary sequelae of SARS-CoV-2 respiratory infections reflect infection severity, innate and adaptive immunity, and respiratory epithelial repair. This study investigated the acute and reparative responses as a function of age and sex in primary human bronchial epithelial (HBE) cultures utilizing a 14-day SARS-CoV-2 infection protocol. SARS-CoV-2 infection peaked at 3 days post-infection (dpi) with an ∼ 2 log titer suppression at 14 dpi. SARS-CoV-2 infection induced interferon, interferon-induced gene, and cell damage responses. No age- or sex-dependent effects on SARS-CoV-2 infection were detected. Airway epithelia repaired to an abnormal mucus metaplastic/inflammatory state that reflected potentially beneficial and adverse consequences at 14 dpi. Repair processes were infection severity-dependent, not sex-dependent, and were more robust in young donor cultures. Analyses of long-COVID subjects with persistent pulmonary fibrosis or persistent bronchitic airway diseases exhibited expression of HBE 14 dpi failed repair gene signatures, including ISG gene signatures. Human airway epithelial repair post-SARS-CoV-2 is prolonged and incomplete in vitro over 14 days, and persistently abnormal repair may contribute to phenotypes of people with long-COVID pulmonary syndrome.

  PublisherDOI

• Differential Mucociliary Clearance and Immune Response in House-Dust-Mite (HDM) Sensitized Mice Following SARS-CoV-2 and Sendai Virus Infections

  American Journal of Respiratory and Critical Care Medicine · 2025-05-01

  articleSenior author

  Abstract RATIONALE: Asthma and respiratory viral infections interact uniquely, impacting disease outcomes and immune responses. Using a mouse model of asthma with house-dust-mite (HDM) sensitization, we investigated the effects of SARS-CoV-2 and Sendai virus (SeV) infections, comparing HDM-sensitized animals with non-treated (NT) littermates. HDM-sensitized mice demonstrated lower viral loads for both SARS-CoV-2 and SeV; however, the mechanisms for viral clearance differed. METHODS: We performed histological observations, immunohistochemistry, RNA in situ hybridization (RNA-ISH), and bulk RNA sequencing to analyze immune and histopathological responses across groups. RESULTS: HDM sensitization significantly increased goblet cell numbers. In SeV-infected HDM mice, goblet cells were empty, showing minimal mucus accumulation in the airways, suggesting efficient mucociliary clearance. Conversely, HDM mice infected with SARS-CoV-2, particularly females, exhibited mucus plugging and mucus-laden goblet cells, indicating impaired mucus release and clearance. Ion channel analysis revealed downregulation of Cftr and Slc26a9, alongside upregulation of epithelial sodium channel (ENaC) in SARS-CoV-2-infected mice, suggesting mucus dehydration may impair clearance, especially detrimental in HDM-sensitized mice with excess mucus production. This impairment facilitated viral spread to the alveoli, causing damage and downregulation of alveolar-specific genes. SeV infection, however, was confined to the airways and did not result in distal lung damage, perhaps reflecting enhanced mucus transport that prevented viral spread. Both viruses induced interferon-stimulated gene expression, albeit HDM sensitization selectively dampened the CXCL10 chemokine response to both viruses, indicating altered inflammation profiles in the asthma model. CONCLUSION: Our findings reveal that HDM sensitization modifies the host response to respiratory viruses, with SeV promoting efficient mucus clearance and limited damage, whereas SARS-CoV-2 exacerbates mucus retention and alveolar spread, particularly in HDM-sensitized mice. This study underscores the need to consider underlying asthma in SARS-CoV-2 pathogenesis and highlights distinct mucociliary and immune responses elicited by respiratory viruses in asthmatic versus non-asthmatic hosts.

  PublisherDOI

• 353 Aberrant ileal mucus properties as a driver of DIOS in CF mice

  Journal of Cystic Fibrosis · 2025-10-01

  articleSenior author
  PublisherDOI

• STAT3-Dependent Regulation of CFTR and Ciliogenesis Is Essential for Mucociliary Clearance and Innate Airway Defense in Hyper-IgE Syndrome

  American Journal of Respiratory and Critical Care Medicine · 2025-06-13 · 3 citations

  articleOpen access

  Abstract Rationale Hyper-IgE syndrome (STAT3-HIES), also known as Job’s syndrome, is a rare immunodeficiency disease typically caused by dominant-negative STAT3 mutations. STAT3-HIES is characterized by chronic pulmonary infection and inflammation, suggesting impaired innate host defense. Objectives To identify airway epithelial host defense defects caused by STAT3 mutations that, together with immune dysfunction, contribute to recurrent pulmonary infections in STAT3-HIES. Methods STAT3-HIES sputum was analyzed for biochemical and biophysical properties. STAT3-HIES excised lungs were harvested for histology, and bronchial brush samples were collected for RNA sequencing and in vitro culture. A STAT3-HIES–specific R382W mutation, expressed via lentivirus, and STAT3 knockout (CRISPR/Cas9) were studied in normal human bronchial epithelial cells under basal or inflammatory (IL1β)-stimulated conditions. Effects of STAT3 deficiency on transcriptomics, epithelial ion channel, secretory, antimicrobial, and ciliary functions were assessed. Measurements and Main Results STAT3-HIES sputum showed increased mucus concentration and viscoelasticity. STAT3-HIES excised lungs exhibited mucus obstruction and elevated IL1β expression. STAT3 mutations reduced CFTR mRNA and protein amounts, impaired CFTR-dependent fluid and mucin secretion, suppressed antimicrobial peptide, cytokine, and chemokine expression, and acidified airway surface liquid at baseline and after IL1β exposure. Notably, mutant STAT3 suppressed IL1R1 expression. Furthermore, STAT3 mutations impaired multiciliogenesis by blocking commitment to ciliated cell lineages through inhibition of HES6, leading to defective mucociliary transport. Administration of a γ-secretase inhibitor restored HES6 expression and improved ciliogenesis in STAT3 R382W mutant cells. Conclusions STAT3 dysfunction leads to multicomponent defects in airway epithelial innate defense, which, in conjunction with immune deficiency, contributes to chronic pulmonary infection in STAT3-HIES.

  PublisherOA PDFDOI

• Profiling endogenous airway proteases and antiproteases and modeling proteolytic activation of Influenza HA using in vitro and ex vivo human airway surface liquid samples

  UNC Libraries · 2025-01-29

  articleOpen access

  Imbalance of airway proteases and antiproteases has been implicated in diseases such as COPD and environmental exposures including cigarette smoke and ozone. To initiate infection, endogenous proteases are commandeered by respiratory viruses upon encountering the airway epithelium. The airway proteolytic environment likely contains redundant antiproteases and proteases with diverse catalytic mechanisms, however a proteomic profile of these enzymes and inhibitors in airway samples has not been reported. The objective of this study was to first profile extracellular proteases and antiproteases using human airway epithelial cell cultures and ex vivo nasal epithelial lining fluid (NELF) samples. Secondly, we present an optimized method for probing the proteolytic environment of airway surface liquid samples (in vitro and ex vivo) using fluorogenic peptides modeling the cleavage sites of respiratory viruses. We detected 48 proteases in the apical wash of cultured human nasal epithelial cells (HNECs) (n = 6) and 57 in NELF (n = 13) samples from healthy human subjects using mass-spectrometry based proteomics. Additionally, we detected 29 and 48 antiproteases in the HNEC apical washes and NELF, respectively. We observed large interindividual variability in rate of cleavage of an Influenza H1 peptide in the ex vivo clinical samples. Since protease and antiprotease levels have been found to be altered in the airways of smokers, we compared proteolytic cleavage in ex vivo nasal lavage samples from male/female smokers and non-smokers. There was a statistically significant increase in proteolysis of Influenza H1 in NLF from male smokers compared to female smokers. Furthermore, we measured cleavage of the S1/S2 site of SARS-CoV, SARS-CoV-2, and SARS-CoV-2 Delta peptides in various airway samples, suggesting the method could be used for other viruses of public health relevance. This assay presents a direct and efficient method of evaluating the proteolytic environment of human airway samples in assessment of therapeutic treatment, exposure, or underlying disease.

  PublisherDOI

• Asthma and COVID-19: Unveiling Outcome Disparities and Treatment Impact Based on Distinct Endotypes.

  UNC Libraries · 2024-11-14

  articleOpen access1st authorCorresponding

  RATIONALE: Epidemiologic studies on asthmatics and <em>in vitro</em> data suggest a protective role of T2 inflammation in SARS-CoV-2 infection. OBJECTIVE: Using a large, multisite cohort, we studied clinical outcomes following SARS-CoV-2 infection in multiple asthma endotypes and examined the effects of T2-directed biologics in infected asthmatics.in Methods: The National COVID Cohort Collaborative (N3C) Data Enclave was used to identify and stratify asthmatic patients by endotype to include non-T2 and T2 asthmatics, as well as exposure to T2-directed biologic therapy. We evaluated the risk of hospitalization, invasive mechanical ventilation, and 90-day mortality by endotype and exposure to biologics. RESULTS: For this study, 402,376 patients met inclusion criteria, of which 138,142 (34%) were characterized as non-T2 and 264,234 (66%) as T2 asthmatics, a group further divided into 104,823 (26%) atopic, 84,440 (21%) eosinophilic, and 74,971 (19%) T2-high asthmatic endotypes. Compared to non-T2 asthmatics, atopic and T2-high asthmatics experienced decreased odds of hospitalization, and 90-day mortality. Conversely, eosinophilic asthmatics experienced higher odds of hospitalization, intubation, and 90-day mortality. Exposure to T2-directed biologic therapies did not alter outcomes after propensity score matching. In contrast, maximum eosinophil count and recent systemic corticosteroid use were directly correlated with increased odds of all outcomes. CONCLUSIONS: COVID-19 outcomes differ depending on asthma endotype, with atopic asthmatics experiencing lower odds and eosinophilic asthmatics experiencing higher odds of deleterious outcomes. T2-directed biologic treatment did not alter these outcomes but recent systemic corticosteroid use predisposes all asthmatics patients to adverse outcomes.&nbsp;

  PublisherDOI

• Biochemical and rheological analysis of human colonic culture mucus reveals similarity to gut mucus

  UNC Libraries · 2024-08-14

  articleOpen access
  PublisherDOI

Frequent coauthors

• David B. Hill

  North Carolina State University

  78 shared

• Richard C. Boucher

  University of North Carolina at Chapel Hill

  78 shared

• Matthew R. Markovetz

  University of North Carolina at Chapel Hill

  63 shared

• Cameron B. Morrison

  Lung Institute

  61 shared

• Mehmet Kesımer

  University of North Carolina at Chapel Hill

  52 shared

• Scott H. Randell

  Lung Institute

  44 shared

• Kenichi Okuda

  Lung Institute

  44 shared

• Takafumi Kato

  42 shared

Education

• Ph.D., Toxicology

  University of North Carolina at Chapel Hill

  1990

• M.S., Toxicology

  University of North Carolina at Chapel Hill

  1986

• B.S., Toxicology

  University of North Carolina at Chapel Hill

  1984