Identification of the retinoschisin-binding site on the retinal Na/K-ATPase.


Journal

PloS one
ISSN: 1932-6203
Titre abrégé: PLoS One
Pays: United States
ID NLM: 101285081

Informations de publication

Date de publication:
2019
Historique:
received: 11 09 2018
accepted: 19 04 2019
entrez: 4 5 2019
pubmed: 3 5 2019
medline: 15 1 2020
Statut: epublish

Résumé

X-linked juvenile retinoschisis (XLRS) is a hereditary retinal dystrophy, caused by mutations in the RS1 gene which encodes the secreted protein retinoschisin. In recent years, several molecules have been proposed to interact with retinoschisin, including the retinal Na/K-ATPase, L-voltage gated Ca2+ channels, and specific sugars. We recently showed that the retinal Na/K-ATPase consisting of subunits ATP1A3 and ATP1B2 is essential for anchoring retinoschisin to plasma membranes and identified the glycosylated ATP1B2 subunit as the direct interaction partner for retinoschisin. We now aimed to precisely map the retinoschisin binding domain(s) in ATP1B2. In general, retinoschisin binding was not affected after selective elimination of individual glycosylation sites via site-directed mutagenesis as well as after full enzymatic deglycosylation of ATP1B2. Applying the interface prediction tool PresCont, two putative protein-protein interaction patches ("patch I" and "patch II") consisting each of four hydrophobic amino acid stretches on the ATP1B2 surface were identified. These were consecutively altered by site-directed mutagenesis. Functional assays with the ATP1B2 patch mutants identified patch II and, specifically, the associated amino acid at position 240 (harboring a threonine in ATP1B2) as crucial for retinoschisin binding to ATP1B2. These and previous results led us to suggest an induced-fit binding mechanism for the interaction between retinoschisin and the Na/K-ATPase, which is dependent on threonine 240 in ATP1B2 allowing the accommodation of hyperflexible retinoschisin spikes by the associated protein-protein interaction patch on ATP1B2.

Identifiants

pubmed: 31048931
doi: 10.1371/journal.pone.0216320
pii: PONE-D-18-26568
pmc: PMC6497308
doi:

Substances chimiques

Atp1b2 protein, mouse 0
Cation Transport Proteins 0
Cell Adhesion Molecules 0
Cell Adhesion Molecules, Neuronal 0
Eye Proteins 0
RS1 protein, mouse 0
Adenosine Triphosphatases EC 3.6.1.-
Atp1a3 protein, mouse EC 3.6.1.-
Sodium-Potassium-Exchanging ATPase EC 7.2.2.13

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

e0216320

Déclaration de conflit d'intérêts

The authors have declared that no competing interests exist.

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Auteurs

Karolina Plössl (K)

Institute of Human Genetics, University of Regensburg, Regensburg, Germany.

Kristina Straub (K)

Institute of Biophysics and Physical Biochemistry, University of Regensburg, Regensburg, Germany.

Verena Schmid (V)

Institute of Human Genetics, University of Regensburg, Regensburg, Germany.

Franziska Strunz (F)

Institute of Human Genetics, University of Regensburg, Regensburg, Germany.

Jens Wild (J)

Institute of Clinical Microbiology and Hygiene, University Hospital of Regensburg, Germany, Regensburg, Germany.

Rainer Merkl (R)

Institute of Biophysics and Physical Biochemistry, University of Regensburg, Regensburg, Germany.

Bernhard H F Weber (BHF)

Institute of Human Genetics, University of Regensburg, Regensburg, Germany.

Ulrike Friedrich (U)

Institute of Human Genetics, University of Regensburg, Regensburg, Germany.

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Classifications MeSH