Lymphatic Connexins and Pannexins in Health and Disease

Ehrlich, Avigail; Molica, Filippo; Hautefort, Aurélie; Kwak, Brenda R.

doi:10.3390/ijms22115734

Cited by 7 publications

(6 citation statements)

References 72 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Hematoxylin/Eosin staining on cryosections of Panx1 LECdel tails of male mice suggested increased interstitial fluid content as compared to male Panx1 fl/fl tails (Figure 5c ). Thus, we measured the tail diameter at two different locations, that is, 1 cm from the basis and 4 cm from the tip, in male and female Panx1 LECdel or control Panx1 fl/fl mice (Ehrlich et al., 2021 ). In agreement with the above results, only male Panx1 LECdel mice exhibited larger tail diameters at both locations (see Figure 5e for the results at 1 cm from the tail tips), pointing to a tail fluid accumulation only in males but not in female mice (Figure 5d ) upon Panx1 deletion from LECs.…”

Section: Resultsmentioning

confidence: 99%

“…Panx1 is part of a glycoprotein family, along with Panx2 and Panx3. Panx1 proteins form heptameric membrane channels, enabling the passage of small signaling molecules, including ATP, between the intra‐ and extra‐cellular space (Ehrlich et al., 2021 ). While Panx2 and Panx3 display distinct expression patterns, Panx1 is ubiquitously expressed, including in LECs (Boucher et al., 2018 ; Molica et al., 2017 ).…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Pannexin1 deletion in lymphatic endothelium affects lymphatic function in a sex‐dependent manner

Ehrlich,

Pelli,

Pick

et al. 2024

Physiological Reports

Self Cite

View full text Add to dashboard Cite

The lymphatic network of capillaries and collecting vessels ensures tissue fluid homeostasis, absorption of dietary fats and trafficking of immune cells. Pannexin1 (Panx1) channels allow for the passage of ions and small metabolites between the cytosol and extracellular environment. Panx1 channels regulate the pathophysiological function of several tissues in a sex‐dependent manner. Here, we studied the role of Panx1 in lymphatic function, and potential sex‐dependent differences therein, in Prox1‐CreERT2Panx1fl/fl and Panx1fl/fl control mice. Panx1 expression was higher in lymphatic endothelial cells (LECs) of male mice. Lymphatic vessel morphology was not affected in Prox1‐CreERT2Panx1fl/fl male and female mice. Lymphatic drainage was decreased by 25% in male Prox1‐CreERT2Panx1fl/fl mice, but was similar in females of both genotypes. Accordingly, only male Prox1‐CreERT2Panx1fl/fl mice exhibited tail swelling, pointing to interstitial fluid accumulation in males upon Panx1 deletion in LECs. Moreover, serum triglyceride and free fatty acid levels raised less in Prox1‐CreERT2Panx1fl/fl mice of both sexes in an oral lipid tolerance test. Finally, the percentage of migratory dendritic cells arriving in draining lymph nodes was increased in Prox1‐CreERT2Panx1fl/fl female mice, but was comparable between male mice of both genotypes. Our results point to a LEC‐specific role for Panx1 in the functions of the lymphatic system.

show abstract

Section: Resultsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Pannexin1 deletion in lymphatic endothelium affects lymphatic function in a sex‐dependent manner

Ehrlich,

Pelli,

Pick

et al. 2024

Physiological Reports

Self Cite

View full text Add to dashboard Cite

show abstract

“…The structural abnormalities of lymphangitic vessels with lymphatic endothelial cells lacking discontinuous intercellular junctions normally found in initial lymphatics, which were necessary for efficient fluid entry, was detected in the initial lymphatics at sites of inflammation [ 31 ]. In addition, mutations or functional defects affecting the lymphatic vessels [ 32 , 33 ] may further predispose to impaired pulmonary lymphatic circulation.…”

Section: Discussionmentioning

confidence: 99%

Plastic Bronchitis—A Serious Rare Complication Affecting Children Only after Fontan Procedure?

Pałyga-Bysiecka

Polewczyk

Kołodziej

et al. 2021

JCM

View full text Add to dashboard Cite

Background: Plastic bronchitis (PB) may occur not only in children following palliative Fontan procedure but also in those without underlying heart disease. We aim to assess the clinical course, therapeutic measures, outcome, and follow-up of PB in children with congenital heart disease (CHD) and children without cardiac problems. Methods: This retrospective case series assessed children with PB admitted to hospital between 2015 and 2019. Parents or guardians of patients were contacted by e-mail or telephone between September 2017 and June 2019 to enquiry about recurrence of PB and strategy of treatment. The diagnosis of PB was based on the expectoration (spontaneous or during bronchoscopy) of endobronchial plugs. Results: This study delineated the clinical, histological, and laboratory features of plastic bronchitis in children following Fontan procedure (Group A) and in those without heart defects (Group B, non-CHD children). The main symptoms were cough accompanied by dyspnea, and hypoxemia with a decrease in oxygen saturation, often leading to acute respiratory failure. In children with CHD, the first episode of PB occurred at a relatively young age. Although chronic, i.e., lasting more than 3 weeks, inhaled therapy was implemented in both groups of patients, the recurrences of PB were observed. The mean time to PB recurrence after the first episode in Group A was longer than that in Group B (1.47 vs. 0.265 years, p = 0.2035). There was no re-episode with recurrence of PB in 3 cases out of 10 in total in Group A (30%) and 1 case out of 4 in total in Group B (25%). While the majority of children in Group A usually developed bronchial casts on the right side, the patients in Group B (without CHD) suffered from bronchial casts located only on the left side. Conclusions: Despite many similarities, clinical, histological, and laboratory studies in the children with plastic bronchitis after Fontan’s surgery and in children without heart defects suggest that there are differences in the course of the disease in patients without CHD, such as a more advanced age of the first episode of PB, the location of plastic casts on the left side, and a stronger role of inflammatory factors and mechanisms. Further research is needed to understand the pathophysiology of PB and choose the most appropriate therapy.

show abstract

“…There is no known overlap between mutations causing CNS disease and those causing lymphedema. Readers interested in further information on the role of GJC2 and other connexins in lymphatic function and lymphedema are referred to a recent review on this topic [ 120 ].…”

Section: Manifestations Of Gjc2 Mutationsmentioning

confidence: 99%

Mechanisms of Diseases Associated with Mutation in GJC2/Connexin 47

Abrams

2023

Biomolecules

View full text Add to dashboard Cite

Connexins are members of a family of integral membrane proteins that provide a pathway for both electrical and metabolic coupling between cells. Astroglia express connexin 30 (Cx30)-GJB6 and Cx43-GJA1, while oligodendroglia express Cx29/Cx31.3-GJC3, Cx32-GJB1, and Cx47-GJC2. Connexins organize into hexameric hemichannels (homomeric if all subunits are identical or heteromeric if one or more differs). Hemichannels from one cell then form cell-cell channels with a hemichannel from an apposed cell. (These are termed homotypic if the hemichannels are identical and heterotypic if the hemichannels differ). Oligodendrocytes couple to each other through Cx32/Cx32 or Cx47/Cx47 homotypic channels and they couple to astrocytes via Cx32/Cx30 or Cx47/Cx43 heterotypic channels. Astrocytes couple via Cx30/Cx30 and Cx43/Cx43 homotypic channels. Though Cx32 and Cx47 may be expressed in the same cells, all available data suggest that Cx32 and Cx47 cannot interact heteromerically. Animal models wherein one or in some cases two different CNS glial connexins have been deleted have helped to clarify the role of these molecules in CNS function. Mutations in a number of different CNS glial connexin genes cause human disease. Mutations in GJC2 lead to three distinct phenotypes, Pelizaeus Merzbacher like disease, hereditary spastic paraparesis (SPG44) and subclinical leukodystrophy.

show abstract

Lymphatic Connexins and Pannexins in Health and Disease

Cited by 7 publications

References 72 publications

Pannexin1 deletion in lymphatic endothelium affects lymphatic function in a sex‐dependent manner

Pannexin1 deletion in lymphatic endothelium affects lymphatic function in a sex‐dependent manner

Plastic Bronchitis—A Serious Rare Complication Affecting Children Only after Fontan Procedure?

Mechanisms of Diseases Associated with Mutation in GJC2/Connexin 47

Contact Info

Product

Resources

About