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Article ; Online: Discoidin domain receptor-2 enhances secondary alveolar septation in mice by activating integrins and modifying focal adhesions.

McGowan, Stephen E

American journal of physiology. Lung cellular and molecular physiology

2023 Volume 324, Issue 3, Page(s) L307–L324

Abstract: The extracellular matrix (ECM) of the pulmonary parenchyma must maintain the structural relationships among resident cells during the constant distortion imposed by respiration. This dictates that both the ECM and cells adapt to changes in shape, while ... ...

Abstract	The extracellular matrix (ECM) of the pulmonary parenchyma must maintain the structural relationships among resident cells during the constant distortion imposed by respiration. This dictates that both the ECM and cells adapt to changes in shape, while retaining their attachment. Membrane-associated integrins and discoidin domain receptors (DDR) bind collagen and transmit signals to the cellular cytoskeleton. Although the contributions of DDR2 to collagen deposition and remodeling during osseous development are evident, it is unclear how DDR2 contributes to lung development. Using mice (
MeSH term(s)	Mice ; Animals ; Discoidin Domain Receptor 2/metabolism ; Integrins/metabolism ; Focal Adhesions/metabolism ; Discoidin Domain Receptors ; Collagen/metabolism ; Lung/metabolism
Chemical Substances	Discoidin Domain Receptor 2 (EC 2.7.10.1) ; Integrins ; Discoidin Domain Receptors (EC 2.7.10.1) ; Collagen (9007-34-5)
Language	English
Publishing date	2023-01-31
Publishing country	United States
Document type	Journal Article ; Research Support, U.S. Gov't, Non-P.H.S.
ZDB-ID	1013184-x
ISSN	1522-1504 ; 1040-0605
ISSN (online)	1522-1504
ISSN	1040-0605
DOI	10.1152/ajplung.00169.2022
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Zs.A 2749: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (2.OG) ab Jg. 2022: Lesesaal (EG)

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Article ; Online: The lipofibroblast: more than a lipid-storage depot.

McGowan, Stephen E

American journal of physiology. Lung cellular and molecular physiology

2019 Volume 316, Issue 5, Page(s) L869–L871

MeSH term(s)	Lipids ; Lung ; Pulmonary Alveoli
Chemical Substances	Lipids
Language	English
Publishing date	2019-03-06
Publishing country	United States
Document type	Journal Article ; Comment
ZDB-ID	1013184-x
ISSN	1522-1504 ; 1040-0605
ISSN (online)	1522-1504
ISSN	1040-0605
DOI	10.1152/ajplung.00109.2019
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Zs.A 2749: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (2.OG) ab Jg. 2022: Lesesaal (EG)

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Article ; Online: Drebrin is Required for Myosin-facilitated Actin Cytoskeletal Remodeling during Pulmonary Alveolar Development.

McGowan, Stephen E / Gilfanov, Nikolas / Chandurkar, Mohanish K / Stiber, Jonathan A / Han, Sangyoon J

American journal of respiratory cell and molecular biology

2024 Volume 70, Issue 4, Page(s) 308–321

Abstract: Alveolar septation increases gas-exchange surface area and requires coordinated cytoskeletal rearrangement in lung fibroblasts (LFs) to balance the demands of contraction and cell migration. We hypothesized that DBN (drebrin), a modulator of the actin ... ...

Abstract	Alveolar septation increases gas-exchange surface area and requires coordinated cytoskeletal rearrangement in lung fibroblasts (LFs) to balance the demands of contraction and cell migration. We hypothesized that DBN (drebrin), a modulator of the actin cytoskeleton in neuronal dendrites, regulates the remodeling of the LF cytoskeleton. Using mice bearing a transgelin-Cre-targeted deletion of Dbn in pulmonary fibroblasts and pericytes, we examined alterations in alveolar septal outgrowth, LF spreading and migration, and actomyosin function. The alveolar surface area and number of alveoli were reduced, whereas alveolar ducts were enlarged, in mice bearing the
MeSH term(s)	Mice ; Animals ; Actins/metabolism ; Neuropeptides/genetics ; Neuropeptides/metabolism ; Lung/metabolism ; Actin Cytoskeleton/metabolism ; Myosin Light Chains/metabolism
Chemical Substances	Actins ; drebrins ; Neuropeptides ; Myosin Light Chains
Language	English
Publishing date	2024-01-25
Publishing country	United States
Document type	Journal Article
ZDB-ID	1025960-0
ISSN	1535-4989 ; 1044-1549
ISSN (online)	1535-4989
ISSN	1044-1549
DOI	10.1165/rcmb.2023-0229OC
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Article ; Online: Neuropilin-1 directs PDGFRα-entry into lung fibroblasts and signaling from very early endosomes.

McGowan, Stephen E / McCoy, Diann M

American journal of physiology. Lung cellular and molecular physiology

2020 Volume 320, Issue 2, Page(s) L179–L192

Abstract: Platelet-derived growth factor receptor-α (PDGFRα) is absolutely required for the development of secondary pulmonary alveolar septa. Our earlier observations indicated that PDGFRα resides intracellularly as well as on the plasma membrane of murine lung ... ...

Abstract	Platelet-derived growth factor receptor-α (PDGFRα) is absolutely required for the development of secondary pulmonary alveolar septa. Our earlier observations indicated that PDGFRα resides intracellularly as well as on the plasma membrane of murine lung fibroblasts (LF). We have examined how neuropilin-1 (Nrp1), a surface receptor without kinase activity, regulates the intracellular trafficking of PDGFRα in LF obtained from mice, some bearing a targeted deletion of Nrp1 in myofibroblasts. Using the proximity ligation assay, we observed that PDGFRα and Nrp1 colocalized in both early antigen-1 (EEA1) containing sorting endosomes and with adaptor protein containing a pleckstrin homology domain and a phosphotyrosine-binding domain-1 (APPL1) in very early endosomes (VEE). These findings were confirmed using live-cell imaging, which demonstrated that recently internalized PDGFRα was observed in Rab5-containing vesicles residing within 100 nm of the plasma membrane. Nrp1 deletion reduced the phosphorylation of Akt (protein kinase B), the major downstream target of PDGFRα, and limited accumulation of inositol-3 phosphates in APPL1-containing endosomes after exposure to PDGFA. PDGFRα co-immunoprecipitated with APPL1, indicating that PDGFRα enters VEE. Targeted deletion of Nrp1 or APPL1-depletion in control LF reduced the activity of an Akt1 biosensor following stimulation with PDGFA. Our findings demonstrate that Nrp1 enhances the entry of PDGFRα into APPL1 containing VEE and that APPL1 enhances PDGFRα signaling. Therefore, Nrp1 promotes endosomal signaling by PDGFRα offering a potential mechanism to explain our prior observation that Nrp1 supports the formation of alveolar ducts and alveoli during secondary septation in mice.
MeSH term(s)	Adaptor Proteins, Signal Transducing/genetics ; Adaptor Proteins, Signal Transducing/metabolism ; Animals ; Endosomes/genetics ; Endosomes/metabolism ; Fibroblasts/cytology ; Fibroblasts/metabolism ; Lung/cytology ; Lung/metabolism ; Mice ; Mice, Knockout ; Neuropilin-1/genetics ; Neuropilin-1/metabolism ; Phosphatidylinositol 3-Kinases/genetics ; Phosphatidylinositol 3-Kinases/metabolism ; Proto-Oncogene Proteins c-akt/genetics ; Proto-Oncogene Proteins c-akt/metabolism ; Receptor, Platelet-Derived Growth Factor beta/genetics ; Receptor, Platelet-Derived Growth Factor beta/metabolism ; Signal Transduction
Chemical Substances	Adaptor Proteins, Signal Transducing ; Appl1 protein, mouse ; Neuropilin-1 (144713-63-3) ; Receptor, Platelet-Derived Growth Factor beta (EC 2.7.10.1) ; Proto-Oncogene Proteins c-akt (EC 2.7.11.1)
Language	English
Publishing date	2020-11-11
Publishing country	United States
Document type	Journal Article ; Research Support, U.S. Gov't, Non-P.H.S.
ZDB-ID	1013184-x
ISSN	1522-1504 ; 1040-0605
ISSN (online)	1522-1504
ISSN	1040-0605
DOI	10.1152/ajplung.00149.2020
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Article ; Online: DNA methylation profiles in the blood of newborn term infants born to mothers with obesity.

Sasaki, Aya / Murphy, Kellie E / Briollais, Laurent / McGowan, Patrick O / Matthews, Stephen G

PloS one

2022 Volume 17, Issue 5, Page(s) e0267946

Abstract: Maternal obesity is an important risk factor for childhood obesity and influences the prevalence of metabolic diseases in offspring. As childhood obesity is influenced by postnatal factors, it is critical to determine whether children born to women with ... ...

Abstract	Maternal obesity is an important risk factor for childhood obesity and influences the prevalence of metabolic diseases in offspring. As childhood obesity is influenced by postnatal factors, it is critical to determine whether children born to women with obesity during pregnancy show alterations that are detectable at birth. Epigenetic mechanisms such as DNA methylation modifications have been proposed to mediate prenatal programming. We investigated DNA methylation signatures in male and female infants from mothers with a normal Body Mass Index (BMI 18.5-24.9 kg/m2) compared to mothers with obesity (BMI≥30 kg/m2). BMI was measured during the first prenatal visit from women recruited into the Ontario Birth Study (OBS) at Mount Sinai Hospital in Toronto, ON, Canada. DNA was extracted from neonatal dried blood spots collected from heel pricks obtained 24 hours after birth at term (total n = 40) from women with a normal BMI and women with obesity matched for parity, age, and neonatal sex. Reduced representation bisulfite sequencing was used to identify genomic loci associated with differentially methylated regions (DMRs) in CpG-dense regions most likely to influence gene regulation. DMRs were predominantly localized to intergenic regions and gene bodies, with only 9% of DMRs localized to promoter regions. Genes associated with DMRs were compared to those from a large publicly available cohort study, the Avon Longitudinal Study of Parents and Children (ALSPAC; total n = 859). Hypergeometric tests revealed a significant overlap in genes associated with DMRs in the OBS and ALSPAC cohorts. PTPRN2, a gene involved in insulin secretion, and MAD1L1, which plays a role in the cell cycle and tumor suppression, contained DMRs in males and females in both cohorts. In males, KEGG pathway analysis revealed significant overrepresentation of genes involved in endocytosis and pathways in cancer, including IGF1R, which was previously shown to respond to diet-induced metabolic stress in animal models and in lymphocytes in the context of childhood obesity. These preliminary findings are consistent with Developmental Origins of Health and Disease paradigm, which posits that adverse prenatal exposures set developmental health trajectories.
MeSH term(s)	Animals ; Child ; Cohort Studies ; DNA Methylation ; Female ; Humans ; Longitudinal Studies ; Male ; Mothers ; Ontario ; Pediatric Obesity/genetics ; Pregnancy
Language	English
Publishing date	2022-05-02
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't ; Research Support, N.I.H., Extramural
ZDB-ID	2267670-3
ISSN	1932-6203 ; 1932-6203
ISSN (online)	1932-6203
ISSN	1932-6203
DOI	10.1371/journal.pone.0267946
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Article ; Online: Design, Synthesis, and Evaluation of Cephamycin-Based Antisporulation Agents targeting

Cun, Wendy Y / Bate, Clara E / Srikhanta, Yogitha N / Hutton, Melanie L / Webb, Chaille T / Revitt-Mills, Sarah A / Lyras, Dena / McGowan, Sheena / Yu, Haibo / Keller, Paul A / Pyne, Stephen G

Journal of medicinal chemistry

2023 Volume 67, Issue 1, Page(s) 450–466

Abstract: With the aim of discovering small molecule inhibitors of the sporulation process ... ...

Abstract	With the aim of discovering small molecule inhibitors of the sporulation process in
MeSH term(s)	Animals ; Mice ; Cephamycins/metabolism ; Clostridioides difficile ; Clostridioides ; Cefotetan/metabolism ; Clostridium Infections ; Spores, Bacterial ; Anti-Bacterial Agents/chemistry ; Bacterial Proteins/metabolism
Chemical Substances	Cephamycins ; Cefotetan (48SPP0PA9Q) ; Anti-Bacterial Agents ; Bacterial Proteins
Language	English
Publishing date	2023-12-19
Publishing country	United States
Document type	Journal Article
ZDB-ID	218133-2
ISSN	1520-4804 ; 0022-2623
ISSN (online)	1520-4804
ISSN	0022-2623
DOI	10.1021/acs.jmedchem.3c01662
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Article ; Online: DNA methylation profiles in the blood of newborn term infants born to mothers with obesity.

Aya Sasaki / Kellie E Murphy / Laurent Briollais / Patrick O McGowan / Stephen G Matthews

PLoS ONE, Vol 17, Iss 5, p e

2022 Volume 0267946

Abstract	Maternal obesity is an important risk factor for childhood obesity and influences the prevalence of metabolic diseases in offspring. As childhood obesity is influenced by postnatal factors, it is critical to determine whether children born to women with obesity during pregnancy show alterations that are detectable at birth. Epigenetic mechanisms such as DNA methylation modifications have been proposed to mediate prenatal programming. We investigated DNA methylation signatures in male and female infants from mothers with a normal Body Mass Index (BMI 18.5-24.9 kg/m2) compared to mothers with obesity (BMI≥30 kg/m2). BMI was measured during the first prenatal visit from women recruited into the Ontario Birth Study (OBS) at Mount Sinai Hospital in Toronto, ON, Canada. DNA was extracted from neonatal dried blood spots collected from heel pricks obtained 24 hours after birth at term (total n = 40) from women with a normal BMI and women with obesity matched for parity, age, and neonatal sex. Reduced representation bisulfite sequencing was used to identify genomic loci associated with differentially methylated regions (DMRs) in CpG-dense regions most likely to influence gene regulation. DMRs were predominantly localized to intergenic regions and gene bodies, with only 9% of DMRs localized to promoter regions. Genes associated with DMRs were compared to those from a large publicly available cohort study, the Avon Longitudinal Study of Parents and Children (ALSPAC; total n = 859). Hypergeometric tests revealed a significant overlap in genes associated with DMRs in the OBS and ALSPAC cohorts. PTPRN2, a gene involved in insulin secretion, and MAD1L1, which plays a role in the cell cycle and tumor suppression, contained DMRs in males and females in both cohorts. In males, KEGG pathway analysis revealed significant overrepresentation of genes involved in endocytosis and pathways in cancer, including IGF1R, which was previously shown to respond to diet-induced metabolic stress in animal models and in lymphocytes in the context ...
Keywords	Medicine ; R ; Science ; Q
Subject code	610
Language	English
Publishing date	2022-01-01T00:00:00Z
Publisher	Public Library of Science (PLoS)
Document type	Article ; Online
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Article ; Online: DNA methylation profiles in the blood of newborn term infants born to mothers with obesity

Aya Sasaki / Kellie E. Murphy / Laurent Briollais / Patrick O. McGowan / Stephen G. Matthews

PLoS ONE, Vol 17, Iss

2022 Volume 5

Abstract	Maternal obesity is an important risk factor for childhood obesity and influences the prevalence of metabolic diseases in offspring. As childhood obesity is influenced by postnatal factors, it is critical to determine whether children born to women with obesity during pregnancy show alterations that are detectable at birth. Epigenetic mechanisms such as DNA methylation modifications have been proposed to mediate prenatal programming. We investigated DNA methylation signatures in male and female infants from mothers with a normal Body Mass Index (BMI 18.5–24.9 kg/m2) compared to mothers with obesity (BMI≥30 kg/m2). BMI was measured during the first prenatal visit from women recruited into the Ontario Birth Study (OBS) at Mount Sinai Hospital in Toronto, ON, Canada. DNA was extracted from neonatal dried blood spots collected from heel pricks obtained 24 hours after birth at term (total n = 40) from women with a normal BMI and women with obesity matched for parity, age, and neonatal sex. Reduced representation bisulfite sequencing was used to identify genomic loci associated with differentially methylated regions (DMRs) in CpG-dense regions most likely to influence gene regulation. DMRs were predominantly localized to intergenic regions and gene bodies, with only 9% of DMRs localized to promoter regions. Genes associated with DMRs were compared to those from a large publicly available cohort study, the Avon Longitudinal Study of Parents and Children (ALSPAC; total n = 859). Hypergeometric tests revealed a significant overlap in genes associated with DMRs in the OBS and ALSPAC cohorts. PTPRN2, a gene involved in insulin secretion, and MAD1L1, which plays a role in the cell cycle and tumor suppression, contained DMRs in males and females in both cohorts. In males, KEGG pathway analysis revealed significant overrepresentation of genes involved in endocytosis and pathways in cancer, including IGF1R, which was previously shown to respond to diet-induced metabolic stress in animal models and in lymphocytes in the context of childhood obesity. These preliminary findings are consistent with Developmental Origins of Health and Disease paradigm, which posits that adverse prenatal exposures set developmental health trajectories.
Keywords	Medicine ; R ; Science ; Q
Subject code	610
Language	English
Publishing date	2022-01-01T00:00:00Z
Publisher	Public Library of Science (PLoS)
Document type	Article ; Online
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Article ; Online: Paracrine cellular and extracellular matrix interactions with mesenchymal progenitors during pulmonary alveolar septation.

McGowan, Stephen E

Birth defects research. Part A, Clinical and molecular teratology

2014 Volume 100, Issue 3, Page(s) 227–239

Abstract: Alveolar development in humans primarily occurs postnatally and requires a carefully orchestrated expansion of distal epithelial and mesenchymal progenitor populations and coordinated differentiation, to create a highly segmented gas-exchange surface. ... ...

Abstract	Alveolar development in humans primarily occurs postnatally and requires a carefully orchestrated expansion of distal epithelial and mesenchymal progenitor populations and coordinated differentiation, to create a highly segmented gas-exchange surface. The regulation of alveolarization normally assimilates cues from paracrine cell-cell, cell-extracellular matrix, and mechanical interactions which are superimposed on cells and the extracellular matrix through phasic respiratory movement. In bronchopulmonary dysplasia, the entire process is precociously initiated when cellular and extracellular components are adapted to the saccular stage where movement and circulation are much more limited. This review focuses on mesenchymal cells (fibroblasts, endothelial cells, and pericytes), and epithelial cells are primarily discussed as sources of growth factor ligands or recipients of ligands produced by mesenchymal cells. Some interstitial fibroblasts differentiate to contractile myofibroblasts, containing a smooth muscle-actin rich cytoskeleton, which connects with tensile and elastic elements in the extracellular matrix, and together comprise a load-bearing network that diffuses mechanical forces during respiration. Other interstitial fibroblasts assimilate neutral lipid droplets, which regulate the differentiation of distal epithelial progenitors and surfactant production by alveolar type 2 cells. Pericytes organize and reinforce the capillary network as it expands to match the coverage of type 1 epithelial cells. Hyperoxia and the mechanical load imposed by positive pressure mechanical ventilation disrupt these paracrine interactions, leaving thickened alveolar walls, airways and arterioles, thereby diminishing gas-exchange surface area. Better understanding of these mechanisms of alveolar septation will lead to more effective treatments to preserve and perhaps augment the surface usual sequence of events that drive alveolarization.
MeSH term(s)	Capillaries/metabolism ; Cell Differentiation/physiology ; Extracellular Matrix/metabolism ; Humans ; Hyperoxia/metabolism ; Mesenchymal Stem Cells/cytology ; Mesenchymal Stem Cells/metabolism ; Paracrine Communication/physiology ; Pulmonary Alveoli/blood supply ; Pulmonary Alveoli/cytology ; Pulmonary Alveoli/growth & development ; Pulmonary Alveoli/metabolism
Language	English
Publishing date	2014-03-17
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	2104792-3
ISSN	1542-0760 ; 1542-0752 ; 1542-9733 ; 1542-975X
ISSN (online)	1542-0760
ISSN	1542-0752 ; 1542-9733 ; 1542-975X
DOI	10.1002/bdra.23230
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Article ; Online: BTB domain mutations perturbing KCTD15 oligomerisation cause a distinctive frontonasal dysplasia syndrome.

Miller, Kerry A / Cruz Walma, David A / Pinkas, Daniel M / Tooze, Rebecca S / Bufton, Joshua C / Richardson, William / Manning, Charlotte E / Hunt, Alice E / Cros, Julien / Hartill, Verity / Parker, Michael J / McGowan, Simon J / Twigg, Stephen R F / Chalk, Rod / Staunton, David / Johnson, David / Wilkie, Andrew O M / Bullock, Alex N

Journal of medical genetics

2024 Volume 61, Issue 5, Page(s) 490–501

Abstract: Introduction: KCTD15: Methods: Exome sequencing was performed on a two-generation family affected by a distinctive phenotype comprising a lipomatous frontonasal malformation, anosmia, cutis aplasia of the scalp and/or sparse hair, and congenital ... ...

Abstract	Introduction: KCTD15 Methods: Exome sequencing was performed on a two-generation family affected by a distinctive phenotype comprising a lipomatous frontonasal malformation, anosmia, cutis aplasia of the scalp and/or sparse hair, and congenital heart disease. Identification of a de novo missense substitution within Results: A heterozygous c.310G>C variant encoding p.(Asp104His) within the BTB domain of Conclusion: BTB domain substitutions in KCTD1 and KCTD15 cause clinically overlapping phenotypes involving craniofacial abnormalities and cutis aplasia. The structural analyses demonstrate that missense substitutions act through a dominant negative mechanism by disrupting the higher order structure of the KCTD15 protein complex.
MeSH term(s)	Humans ; BTB-POZ Domain ; Mutation ; Craniofacial Abnormalities/genetics ; Abnormalities, Multiple ; Mutation, Missense/genetics ; Syndrome ; Ectodermal Dysplasia ; Co-Repressor Proteins/genetics ; Face/abnormalities
Chemical Substances	KCTD1 protein, human ; Co-Repressor Proteins
Language	English
Publishing date	2024-04-19
Publishing country	England
Document type	Journal Article
ZDB-ID	220881-7
ISSN	1468-6244 ; 0022-2593
ISSN (online)	1468-6244
ISSN	0022-2593
DOI	10.1136/jmg-2023-109531
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