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Rafael Aldabe

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Articles 44
Citations 784
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Recent Articles
1.
Llanos-Ardaiz A, Lantero A, Neri L, Mauleon I, Ruiz de Galarreta M, Trigueros-Motos L, et al.
Int J Mol Sci . 2024 Dec; 25(23). PMID: 39684442
Adeno-associated viral (AAV) vector-mediated gene therapy has emerged as a promising alternative to liver transplantation for monogenic metabolic hepatic diseases. AAVs are non-integrative vectors that are maintained primarily as episomes...
2.
Delgado-Rodriguez P, Lamanna-Rama N, Saande C, Aldabe R, Soto-Montenegro M, Munoz-Barrutia A
Commun Biol . 2024 Sep; 7(1):1183. PMID: 39300231
Autosomal Dominant Polycystic Kidney Disease (ADPKD) is the most prevalent kidney genetic disorder, producing structural abnormalities and impaired function. This research investigates its evolution on mouse models, utilizing a combination...
3.
Guedes J, Boyer J, Elurbide J, Carte B, Redeker V, Sago L, et al.
Mol Cell Biol . 2024 Aug; 44(9):358-371. PMID: 39099191
N-terminal acetyltransferase B (NatB) is a major contributor to the N-terminal acetylome and is implicated in several key cellular processes including apoptosis and proteostasis. However, the molecular mechanisms linking NatB-mediated...
4.
Milagros S, de Erenchun P, Guembe M, Carte B, Mendez M, Uribarri A, et al.
J Biol Eng . 2024 May; 18(1):34. PMID: 38745236
Background: Several treatments for genetic diseases utilizing recombinant adeno-associated viruses (AAVs) have recently gained approval. However, the development of a greater number of therapeutic AAVs is constrained by certain limitations....
5.
Camps G, Maestro S, Torella L, Herrero D, Usai C, Bilbao-Arribas M, et al.
PLoS Pathog . 2024 May; 20(5):e1011749. PMID: 38739648
Hepatitis delta virus (HDV) infection represents the most severe form of human viral hepatitis; however, the mechanisms underlying its pathology remain incompletely understood. We recently developed an HDV mouse model...
6.
Lyon G, Longo J, Garcia A, Inusa F, Marchi E, Shi D, et al.
PLoS One . 2024 May; 19(5):e0301328. PMID: 38713657
Amino-terminal (Nt-) acetylation (NTA) is a common protein modification, affecting approximately 80% of all human proteins. The human essential X-linked gene, NAA10, encodes for the enzyme NAA10, which is the...
7.
Maestro S, Gomez-Echarte N, Camps G, Usai C, Olague C, Vales A, et al.
Viruses . 2024 Mar; 16(3). PMID: 38543745
Hepatitis D virus (HDV) infection represents the most severe form of chronic viral hepatitis. We have shown that the delivery of HDV replication-competent genomes to the hepatocytes using adeno-associated virus...
8.
Elurbide J, Carte B, Guedes J, Aldabe R
Int J Mol Sci . 2023 May; 24(10). PMID: 37240070
Alpha-aminoterminal acetyltransferase B (NatB) is a critical enzyme responsible for acetylating the aminoterminal end of proteins, thereby modifying approximately 21% of the proteome. This post-translational modification impacts protein folding, structure,...
9.
Lyon G, Longo J, Garcia A, Inusa F, Marchi E, Shi D, et al.
bioRxiv . 2023 May; PMID: 37163119
Amino-terminal (Nt-) acetylation (NTA) is a common protein modification, affecting approximately 80% of all human proteins. The human essential X-linked gene, , encodes for the enzyme NAA10, which is the...
10.
Bazo A, Lantero A, Mauleon I, Neri L, Poms M, Haberle J, et al.
Int J Mol Sci . 2022 Dec; 23(23). PMID: 36499263
Citrullinemia type I (CTLN1) is a rare autosomal recessive disorder caused by mutations in the gene encoding argininosuccinate synthetase 1 (ASS1) that catalyzes the third step of the urea cycle....