Nanobodies as tools for studying human frataxin biology
20 pages, 10 figures, 1 table
| Autores: | , , , , , , , , , , , , , , , , , |
|---|---|
| Tipo de recurso: | artículo |
| Estado: | Versión publicada |
| Fecha de publicación: | 2026 |
| País: | España |
| Institución: | Consejo Superior de Investigaciones Científicas (CSIC) |
| Repositorio: | DIGITAL.CSIC. Repositorio Institucional del CSIC |
| OAI Identifier: | oai:dnet:digitalcsic_::ebe143c806b58f6f48d87267beb91534 |
| Acceso en línea: | http://hdl.handle.net/10261/430105 https://api.elsevier.com/content/abstract/scopus_id/105029532306 |
| Access Level: | acceso abierto |
| Palabra clave: | http://metadata.un.org/sdg/3 Ensure healthy lives and promote well-being for all at all ages biology Life sciences |
| id |
ES_989b68dcded6a6f42cb3beeb2b17aa89 |
|---|---|
| oai_identifier_str |
oai:dnet:digitalcsic_::ebe143c806b58f6f48d87267beb91534 |
| network_acronym_str |
ES |
| network_name_str |
España |
| repository_id_str |
|
| dc.title.none.fl_str_mv |
Nanobodies as tools for studying human frataxin biology |
| title |
Nanobodies as tools for studying human frataxin biology |
| spellingShingle |
Nanobodies as tools for studying human frataxin biology Pignataro, María Florencia http://metadata.un.org/sdg/3 Ensure healthy lives and promote well-being for all at all ages biology Life sciences |
| title_short |
Nanobodies as tools for studying human frataxin biology |
| title_full |
Nanobodies as tools for studying human frataxin biology |
| title_fullStr |
Nanobodies as tools for studying human frataxin biology |
| title_full_unstemmed |
Nanobodies as tools for studying human frataxin biology |
| title_sort |
Nanobodies as tools for studying human frataxin biology |
| dc.creator.none.fl_str_mv |
Pignataro, María Florencia Fernández, Natalia Brenda Garay-Álvarez, Alba Pavan, María Florencia Molina, Rafael Muñoz, Inés G. Grossi, Julián Noguera, Martín Vila, Antonella García, Augusto E. Gentili, Hernán G. Rodríguez, Naira Antonia Aran, Martín Parreño, Viviana Bok, Marina Hermoso, Juan A. Ibañez, Lorena Itatí Santos, Javier |
| author |
Pignataro, María Florencia |
| author_facet |
Pignataro, María Florencia Fernández, Natalia Brenda Garay-Álvarez, Alba Pavan, María Florencia Molina, Rafael Muñoz, Inés G. Grossi, Julián Noguera, Martín Vila, Antonella García, Augusto E. Gentili, Hernán G. Rodríguez, Naira Antonia Aran, Martín Parreño, Viviana Bok, Marina Hermoso, Juan A. Ibañez, Lorena Itatí Santos, Javier |
| author_role |
author |
| author2 |
Fernández, Natalia Brenda Garay-Álvarez, Alba Pavan, María Florencia Molina, Rafael Muñoz, Inés G. Grossi, Julián Noguera, Martín Vila, Antonella García, Augusto E. Gentili, Hernán G. Rodríguez, Naira Antonia Aran, Martín Parreño, Viviana Bok, Marina Hermoso, Juan A. Ibañez, Lorena Itatí Santos, Javier |
| author2_role |
author author author author author author author author author author author author author author author author author |
| dc.contributor.none.fl_str_mv |
Universidad de Buenos Aires Consejo Nacional de Investigaciones Científicas y Técnicas (Argentina) Friedreich´s Ataxia Research Alliance Ministerio de Ciencia e Innovación (España) ALBA Synchrotron Pignataro, María Florencia [0000-0002-5398-8724] #NODATA# Garay-Álvarez, Alba [0009-0001-6544-7327] Pavan, María Florencia [0009-0007-3646-5749] Molina, Rafael [0000-0002-6443-4989] Muñoz, Inés G. [0000-0001-6732-4059] #NODATA# Noguera, Martín [0000-0001-5868-3834] #NODATA# García, Augusto E. [0000-0001-7668-2050] #NODATA# #NODATA# #NODATA# #NODATA# #NODATA# Hermoso, Juan A. [0000-0002-1862-8950] Ibañez, Lorena Itatí [0000-0002-9393-2004] Santos, Javier [0000-0002-1140-8234] Consejo Superior de Investigaciones Científicas [https://ror.org/02gfc7t72] |
| dc.subject.none.fl_str_mv |
http://metadata.un.org/sdg/3 Ensure healthy lives and promote well-being for all at all ages biology Life sciences |
| topic |
http://metadata.un.org/sdg/3 Ensure healthy lives and promote well-being for all at all ages biology Life sciences |
| description |
20 pages, 10 figures, 1 table |
| publishDate |
2026 |
| dc.date.none.fl_str_mv |
2026 2026 2026 |
| dc.type.none.fl_str_mv |
info:eu-repo/semantics/article http://purl.org/coar/resource_type/c_6501 Publisher's version info:eu-repo/semantics/publishedVersion |
| format |
article |
| status_str |
publishedVersion |
| dc.identifier.none.fl_str_mv |
http://hdl.handle.net/10261/430105 https://api.elsevier.com/content/abstract/scopus_id/105029532306 |
| url |
http://hdl.handle.net/10261/430105 https://api.elsevier.com/content/abstract/scopus_id/105029532306 |
| dc.language.none.fl_str_mv |
Inglés |
| language_invalid_str_mv |
Inglés |
| dc.relation.none.fl_str_mv |
#PLACEHOLDER_PARENT_METADATA_VALUE# #PLACEHOLDER_PARENT_METADATA_VALUE# #PLACEHOLDER_PARENT_METADATA_VALUE# info:eu-repo/grantAgreement/AEI/Plan Estatal de Investigación Científica y Técnica y de Innovación 2017-2020/PID2020-115331GB-I00 info:eu-repo/grantAgreement/AEI/Plan Estatal de Investigación Científica y Técnica y de Innovación 2021-2023/PID2023-153118OB-I00 info:eu-repo/grantAgreement/AEI/Plan Estatal de Investigación Científica y Técnica y de Innovación 2021-2023/PID2022-136750NB-I00 https://doi.org/10.1038/s42003-025-09458-x Sí |
| dc.rights.none.fl_str_mv |
info:eu-repo/semantics/openAccess |
| eu_rights_str_mv |
openAccess |
| dc.publisher.none.fl_str_mv |
Nature Publishing Group |
| publisher.none.fl_str_mv |
Nature Publishing Group |
| dc.source.none.fl_str_mv |
reponame:DIGITAL.CSIC. Repositorio Institucional del CSIC instname:Consejo Superior de Investigaciones Científicas (CSIC) |
| instname_str |
Consejo Superior de Investigaciones Científicas (CSIC) |
| reponame_str |
DIGITAL.CSIC. Repositorio Institucional del CSIC |
| collection |
DIGITAL.CSIC. Repositorio Institucional del CSIC |
| repository.name.fl_str_mv |
|
| repository.mail.fl_str_mv |
|
| _version_ |
1869414206806163456 |
| spelling |
Nanobodies as tools for studying human frataxin biologyPignataro, María FlorenciaFernández, Natalia BrendaGaray-Álvarez, AlbaPavan, María FlorenciaMolina, RafaelMuñoz, Inés G.Grossi, JuliánNoguera, MartínVila, AntonellaGarcía, Augusto E.Gentili, Hernán G.Rodríguez, Naira AntoniaAran, MartínParreño, VivianaBok, MarinaHermoso, Juan A.Ibañez, Lorena ItatíSantos, Javierhttp://metadata.un.org/sdg/3Ensure healthy lives and promote well-being for all at all agesbiologyLife sciences20 pages, 10 figures, 1 tableIron-sulfur clusters are essential cofactors for the accurate cellular function of many proteins. In eukaryotic cells, the biogenesis of most iron-sulfur clusters occurs in the mitochondria and involves the action of the Cys desulfurase supercomplex, which is activated by the protein frataxin (FXN). The decrease of FXN expression and/or function results in Friedreich's ataxia (FRDA).In this work, several nanobodies specific to human FXN were selected via phage display, demonstrating a wide range of effects on Cys desulfurase activity and a strong interaction with FXN. Nanobody interaction stabilized wild-type and FRDA-related FXN variants in vitro. FXN-nanobody complexes were characterized by NMR, SAXS, and X-ray crystallography. Additionally, Nanobody expression was studied in human cells. The subcellular localization, direct interaction with FXN by in situ proximity ligation assay, effect on cell viability, Fe-S-dependent enzymatic activities, and oxygen consumption rates were analyzed. Significantly, nanobody expression did not alter these key metabolic variables, suggesting that the interaction with FXN did not disrupt the pathway.As a whole, our results suggest that nanobodies can serve as binding partners for mitochondrial FXN. However, the specific effect of the nanobodies on the conformational stability of FRDA-related FXN variants in cells should be investigated.This work was supported by Universidad de Buenos Aires UBACyT20020190100338BA, CONICET, and Friedreich´s Ataxia Research Alliance (FARA, grant 2023-2025). The work in Spain was funded by the Spanish Ministry of Science and Innovation by grants PID2020-115331GB-100 and PID2023-153118OB-100 to J.A.H., and PID2022-136750NB-I00 to R.M. We gratefully acknowledge the staff of the XALOC beamline at the ALBA Synchrotron in Barcelona for their valuable assistance with data collection. We would also like to thank Lic. Alejo Cantoia, Dr. Gernán Rosano, and Dr. Eduardo Ceccarelli from the UEM-IBR (Mass spectrometry unit of the Instituto de Biología Molecular y Celular de Rosario, Argentina) for technical assistance and data processing of LC-MS experiments.Peer reviewedNature Publishing GroupUniversidad de Buenos AiresConsejo Nacional de Investigaciones Científicas y Técnicas (Argentina)Friedreich´s Ataxia Research AllianceMinisterio de Ciencia e Innovación (España)ALBA SynchrotronPignataro, María Florencia [0000-0002-5398-8724]#NODATA#Garay-Álvarez, Alba [0009-0001-6544-7327]Pavan, María Florencia [0009-0007-3646-5749]Molina, Rafael [0000-0002-6443-4989]Muñoz, Inés G. [0000-0001-6732-4059]#NODATA#Noguera, Martín [0000-0001-5868-3834]#NODATA#García, Augusto E. [0000-0001-7668-2050]#NODATA##NODATA##NODATA##NODATA##NODATA#Hermoso, Juan A. [0000-0002-1862-8950]Ibañez, Lorena Itatí [0000-0002-9393-2004]Santos, Javier [0000-0002-1140-8234]Consejo Superior de Investigaciones Científicas [https://ror.org/02gfc7t72]202620262026info:eu-repo/semantics/articlehttp://purl.org/coar/resource_type/c_6501Publisher's versioninfo:eu-repo/semantics/publishedVersionhttp://hdl.handle.net/10261/430105https://api.elsevier.com/content/abstract/scopus_id/105029532306reponame:DIGITAL.CSIC. Repositorio Institucional del CSICinstname:Consejo Superior de Investigaciones Científicas (CSIC)Inglés#PLACEHOLDER_PARENT_METADATA_VALUE##PLACEHOLDER_PARENT_METADATA_VALUE##PLACEHOLDER_PARENT_METADATA_VALUE#info:eu-repo/grantAgreement/AEI/Plan Estatal de Investigación Científica y Técnica y de Innovación 2017-2020/PID2020-115331GB-I00info:eu-repo/grantAgreement/AEI/Plan Estatal de Investigación Científica y Técnica y de Innovación 2021-2023/PID2023-153118OB-I00info:eu-repo/grantAgreement/AEI/Plan Estatal de Investigación Científica y Técnica y de Innovación 2021-2023/PID2022-136750NB-I00https://doi.org/10.1038/s42003-025-09458-xSíinfo:eu-repo/semantics/openAccessoai:dnet:digitalcsic_::ebe143c806b58f6f48d87267beb915342026-05-22T06:33:51Z |
| score |
15,812429 |