Differential effects of adenylyl cyclase-protein kinase A cascade on shear-induced changes of sickle cell deformability.


Journal

Clinical hemorheology and microcirculation
ISSN: 1875-8622
Titre abrégé: Clin Hemorheol Microcirc
Pays: Netherlands
ID NLM: 9709206

Informations de publication

Date de publication:
2019
Historique:
pubmed: 16 7 2019
medline: 31 3 2020
entrez: 16 7 2019
Statut: ppublish

Résumé

Erythrocyte deformability is impaired in sickle cell disease (SCD). The regulation of cytoskeletal protein organization plays a key role in erythrocyte deformability. The activation of adenylyl cyclase (AC)/cAMP/Protein kinase A (PKA) signaling pathway was associated with increased deformability in healthy erythrocytes, however the role of this pathway in SCD is unknown. We evaluated mechanical responses of sickle red blood cells under physiological levels of shear stress and the possible link between their deformability and AC/cAMP/PKA signaling pathway. The shearing of sickle red blood cells at physiological level (5 Pa) and the measurement of deformability were performed by a laser assisted optical rotational cell analyzer (LORRCA). Red blood cell deformability increased of 2.5-6.5% by blocking the activity of phosphodiesterase with Pentoxifylline (10μM) (p < 0.05). The inhibition of AC with SQ22536 (100μM) produced more significant rise in deformability (+4.8-12%, p < 0.01). No significant change was observed by the inhibition of PKA with H89 (10μM). Pentoxifylline and SQ22536 increased the deformability of sickle red blood cells under fluid shear stress. Modulation of the AC/cAMP/PKA pathway could have the potential to be an effective therapeutic approach for SCD through shear-induced improvements of RBC deformability.

Sections du résumé

BACKGROUND BACKGROUND
Erythrocyte deformability is impaired in sickle cell disease (SCD). The regulation of cytoskeletal protein organization plays a key role in erythrocyte deformability. The activation of adenylyl cyclase (AC)/cAMP/Protein kinase A (PKA) signaling pathway was associated with increased deformability in healthy erythrocytes, however the role of this pathway in SCD is unknown.
OBJECTIVE OBJECTIVE
We evaluated mechanical responses of sickle red blood cells under physiological levels of shear stress and the possible link between their deformability and AC/cAMP/PKA signaling pathway.
METHODS METHODS
The shearing of sickle red blood cells at physiological level (5 Pa) and the measurement of deformability were performed by a laser assisted optical rotational cell analyzer (LORRCA).
RESULTS RESULTS
Red blood cell deformability increased of 2.5-6.5% by blocking the activity of phosphodiesterase with Pentoxifylline (10μM) (p < 0.05). The inhibition of AC with SQ22536 (100μM) produced more significant rise in deformability (+4.8-12%, p < 0.01). No significant change was observed by the inhibition of PKA with H89 (10μM).
CONCLUSION CONCLUSIONS
Pentoxifylline and SQ22536 increased the deformability of sickle red blood cells under fluid shear stress. Modulation of the AC/cAMP/PKA pathway could have the potential to be an effective therapeutic approach for SCD through shear-induced improvements of RBC deformability.

Identifiants

pubmed: 31306111
pii: CH190563
doi: 10.3233/CH-190563
doi:

Substances chimiques

Cyclic AMP-Dependent Protein Kinases EC 2.7.11.11
Adenylyl Cyclases EC 4.6.1.1

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

531-543

Auteurs

Elif Ugurel (E)

Department of Physiology, Koç University School of Medicine, Sariyer, Istanbul, Turkey.

Philippe Connes (P)

Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Team « Vascular Biology and Red Blood Cell », Université Claude Bernard Lyon 1, Lyon, France.
Laboratoire d'Excellence du Globule Rouge (Labex GR-Ex), PRES Sorbonne, Paris, France.
Institut Universitaire de France (IUF), Paris, France.

Gokce Yavas (G)

Department of Physiology, Koç University School of Medicine, Sariyer, Istanbul, Turkey.

Buse Eglenen (B)

Department of Physiology, Koç University School of Medicine, Sariyer, Istanbul, Turkey.

Mine Turkay (M)

Department of Physiology, Koç University School of Medicine, Sariyer, Istanbul, Turkey.

Ali Cenk Aksu (AC)

Department of Physiology, Koç University School of Medicine, Sariyer, Istanbul, Turkey.

Celine Renoux (C)

Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Team « Vascular Biology and Red Blood Cell », Université Claude Bernard Lyon 1, Lyon, France.
Laboratoire d'Excellence du Globule Rouge (Labex GR-Ex), PRES Sorbonne, Paris, France.
UF de biochimie des pathologies érythrocytaires, Centre de Biologie Est, Hospices Civils de Lyon, Lyon, France.

Philippe Joly (P)

Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Team « Vascular Biology and Red Blood Cell », Université Claude Bernard Lyon 1, Lyon, France.
Laboratoire d'Excellence du Globule Rouge (Labex GR-Ex), PRES Sorbonne, Paris, France.
UF de biochimie des pathologies érythrocytaires, Centre de Biologie Est, Hospices Civils de Lyon, Lyon, France.

Alexandra Gauthier (A)

Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Team « Vascular Biology and Red Blood Cell », Université Claude Bernard Lyon 1, Lyon, France.
Laboratoire d'Excellence du Globule Rouge (Labex GR-Ex), PRES Sorbonne, Paris, France.
Institut d'hématologie et d'oncologie pédiatrique (IHOP), Hospices Civils de Lyon, Lyon, France.

Arnaud Hot (A)

Clinique de Médecine Ambulatoire/Hématologie Hôpital Edouard Herriot, Lyon, Lyon, France.

Yves Bertrand (Y)

Institut d'hématologie et d'oncologie pédiatrique (IHOP), Hospices Civils de Lyon, Lyon, France.

Giovanna Cannas (G)

Laboratoire Interuniversitaire de Biologie de la Motricité (LIBM) EA7424, Team « Vascular Biology and Red Blood Cell », Université Claude Bernard Lyon 1, Lyon, France.
Laboratoire d'Excellence du Globule Rouge (Labex GR-Ex), PRES Sorbonne, Paris, France.
Clinique de Médecine Ambulatoire/Hématologie Hôpital Edouard Herriot, Lyon, Lyon, France.

Ozlem Yalcin (O)

Department of Physiology, Koç University School of Medicine, Sariyer, Istanbul, Turkey.

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Classifications MeSH