BPIFB1 (LPLUNC1) is upregulated in cystic fibrosis lung disease

Lynne Bingle, Kirsty Wilson, Maslinda Musa, Bianca Araujo, Doris Rassl, William A. Wallace, Elizabeth E. LeClair, Thais Mauad, Zhe Zhou, Marcus A. Mall, Colin D. Bingle

Research output: Contribution to journalArticleResearchpeer-review

15 Citations (Scopus)

Abstract

Although the biology the PLUNC (recently renamed BPI fold, BPIF) family of secreted proteins is poorly understood, multiple array based studies have suggested that some are differentially expressed in lung diseases. We have examined the expression of BPIFB1 (LPLUNC1), the prototypic two-domain containing family member, in lungs from CF patients and in mouse models of CF lung disease. BPIFB1 was localized in CF lung samples along with BPIFA1, MUC5AC, CD68 and NE and directly compared to histologically normal lung tissues and that of bacterial pneumonia. We generated novel antibodies to mouse BPIF proteins to conduct similar studies on ENaC transgenic (ENaC-Tg) mice, a model for CF-like lung disease. Small airways in CF demonstrated marked epithelial staining of BPIFB1 in goblet cells but staining was absent from alveolar regions. BPIFA1 and BPIFB1 were not co-localised in the diseased lungs. In ENaC-Tg mice there was strong staining of both proteins in the airways and luminal contents. This was most marked for BPIFB1 and was noted within 2 weeks of birth. The two proteins were present in distinct cells within epithelium. BPIFB1 was readily detected in BAL from ENaC-Tg mice but was absent from wild-type mice. Alterations in the expression of BPIF proteins is associated with CF lung disease in humans and mice. It is unclear if this elevation of protein production, which results from phenotypic alteration of the cells within the diseased epithelium, plays a role in the pathogenesis of the disease.

Original languageEnglish
Pages (from-to)749-758
Number of pages10
JournalHistochemistry and Cell Biology
Volume138
Issue number5
DOIs
Publication statusPublished - Nov 2012
Externally publishedYes

Keywords

  • Airway
  • BPIFB1
  • Cystic fibrosis
  • Immunohistochemistry
  • LPLUNC1
  • Mouse models

Cite this

Bingle, L., Wilson, K., Musa, M., Araujo, B., Rassl, D., Wallace, W. A., ... Bingle, C. D. (2012). BPIFB1 (LPLUNC1) is upregulated in cystic fibrosis lung disease. Histochemistry and Cell Biology, 138(5), 749-758. https://doi.org/10.1007/s00418-012-0990-8
Bingle, Lynne ; Wilson, Kirsty ; Musa, Maslinda ; Araujo, Bianca ; Rassl, Doris ; Wallace, William A. ; LeClair, Elizabeth E. ; Mauad, Thais ; Zhou, Zhe ; Mall, Marcus A. ; Bingle, Colin D. / BPIFB1 (LPLUNC1) is upregulated in cystic fibrosis lung disease. In: Histochemistry and Cell Biology. 2012 ; Vol. 138, No. 5. pp. 749-758.
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abstract = "Although the biology the PLUNC (recently renamed BPI fold, BPIF) family of secreted proteins is poorly understood, multiple array based studies have suggested that some are differentially expressed in lung diseases. We have examined the expression of BPIFB1 (LPLUNC1), the prototypic two-domain containing family member, in lungs from CF patients and in mouse models of CF lung disease. BPIFB1 was localized in CF lung samples along with BPIFA1, MUC5AC, CD68 and NE and directly compared to histologically normal lung tissues and that of bacterial pneumonia. We generated novel antibodies to mouse BPIF proteins to conduct similar studies on ENaC transgenic (ENaC-Tg) mice, a model for CF-like lung disease. Small airways in CF demonstrated marked epithelial staining of BPIFB1 in goblet cells but staining was absent from alveolar regions. BPIFA1 and BPIFB1 were not co-localised in the diseased lungs. In ENaC-Tg mice there was strong staining of both proteins in the airways and luminal contents. This was most marked for BPIFB1 and was noted within 2 weeks of birth. The two proteins were present in distinct cells within epithelium. BPIFB1 was readily detected in BAL from ENaC-Tg mice but was absent from wild-type mice. Alterations in the expression of BPIF proteins is associated with CF lung disease in humans and mice. It is unclear if this elevation of protein production, which results from phenotypic alteration of the cells within the diseased epithelium, plays a role in the pathogenesis of the disease.",
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Bingle, L, Wilson, K, Musa, M, Araujo, B, Rassl, D, Wallace, WA, LeClair, EE, Mauad, T, Zhou, Z, Mall, MA & Bingle, CD 2012, 'BPIFB1 (LPLUNC1) is upregulated in cystic fibrosis lung disease', Histochemistry and Cell Biology, vol. 138, no. 5, pp. 749-758. https://doi.org/10.1007/s00418-012-0990-8

BPIFB1 (LPLUNC1) is upregulated in cystic fibrosis lung disease. / Bingle, Lynne; Wilson, Kirsty; Musa, Maslinda; Araujo, Bianca; Rassl, Doris; Wallace, William A.; LeClair, Elizabeth E.; Mauad, Thais; Zhou, Zhe; Mall, Marcus A.; Bingle, Colin D.

In: Histochemistry and Cell Biology, Vol. 138, No. 5, 11.2012, p. 749-758.

Research output: Contribution to journalArticleResearchpeer-review

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T1 - BPIFB1 (LPLUNC1) is upregulated in cystic fibrosis lung disease

AU - Bingle, Lynne

AU - Wilson, Kirsty

AU - Musa, Maslinda

AU - Araujo, Bianca

AU - Rassl, Doris

AU - Wallace, William A.

AU - LeClair, Elizabeth E.

AU - Mauad, Thais

AU - Zhou, Zhe

AU - Mall, Marcus A.

AU - Bingle, Colin D.

PY - 2012/11

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KW - BPIFB1

KW - Cystic fibrosis

KW - Immunohistochemistry

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Bingle L, Wilson K, Musa M, Araujo B, Rassl D, Wallace WA et al. BPIFB1 (LPLUNC1) is upregulated in cystic fibrosis lung disease. Histochemistry and Cell Biology. 2012 Nov;138(5):749-758. https://doi.org/10.1007/s00418-012-0990-8