Erik J Bonten
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Explore the profile of Erik J Bonten including associated specialties, affiliations and a list of published articles.
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19
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621
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Recent Articles
11.
Bonten E, Annunziata I, dAzzo A
Cell Mol Life Sci
. 2013 Dec;
71(11):2017-32.
PMID: 24337808
The ubiquitous distribution of lysosomes and their heterogeneous protein composition reflects the versatility of these organelles in maintaining cell homeostasis and their importance in tissue differentiation and remodeling. In lysosomes,...
12.
Bonten E, Yogalingam G, Hu H, Gomero E, van de Vlekkert D, dAzzo A
Biochim Biophys Acta
. 2013 Jun;
1832(10):1784-92.
PMID: 23770387
The lysosomal storage disease sialidosis is caused by a primary deficiency of the sialidase N-acetyl-α-neuraminidase-1 (NEU1). Patients with type I sialidosis develop an attenuated, non-neuropathic form of the disease also...
13.
Stamatos N, Carubelli I, van de Vlekkert D, Bonten E, Papini N, Feng C, et al.
J Leukoc Biol
. 2010 Sep;
88(6):1227-39.
PMID: 20826611
Removal of sialic acid from glycoconjugates on the surface of monocytes enhances their response to bacterial LPS. We tested the hypothesis that endogenous sialidase activity creates a permissive state for...
14.
Zanoteli E, van de Vlekkert D, Bonten E, Hu H, Mann L, Gomero E, et al.
Biochim Biophys Acta
. 2010 Apr;
1802(7-8):659-72.
PMID: 20388541
Neuraminidase 1 (NEU1) regulates the catabolism of sialoglycoconjugates in lysosomes. Congenital NEU1 deficiency in children is the basis of sialidosis, a severe neurosomatic disorder in which patients experience a broad...
15.
Bonten E, Campos Y, Zaitsev V, Nourse A, Waddell B, Lewis W, et al.
J Biol Chem
. 2009 Aug;
284(41):28430-28441.
PMID: 19666471
Lysosomal neuraminidase-1 (NEU1) forms a multienzyme complex with beta-galactosidase and protective protein/cathepsin A (PPCA). Because of its association with PPCA, which acts as a molecular chaperone, NEU1 is transported to...
16.
Yogalingam G, Bonten E, van de Vlekkert D, Hu H, Moshiach S, Connell S, et al.
Dev Cell
. 2008 Jul;
15(1):74-86.
PMID: 18606142
Lysosomal exocytosis is a Ca2+-regulated mechanism that involves proteins responsible for cytoskeletal attachment and fusion of lysosomes with the plasma membrane. However, whether luminal lysosomal enzymes contribute to this process...
17.
Wang D, Bonten E, Yogalingam G, Mann L, dAzzo A
Mol Genet Metab
. 2005 Jun;
85(3):181-9.
PMID: 15979029
Given the success of enzyme replacement therapy (ERT) in treating the systemic manifestations in a number of lysosomal storage disorders (LSDs), we evaluated the effect of ERT on the mouse...
18.
Bonten E, Wang D, Toy J, Mann L, Mignardot A, Yogalingam G, et al.
FASEB J
. 2004 Apr;
18(9):971-3.
PMID: 15084520
Lysosomal storage diseases (LSDs) are monogenic disorders of metabolism caused by deficiency of hydrolytic enzymes. In several LSDs, cells of the reticuloendothelial (RE) system are the primary targets of the...
19.
Cuervo A, Mann L, Bonten E, dAzzo A, Dice J
EMBO J
. 2002 Dec;
22(1):47-59.
PMID: 12505983
Protective protein/cathepsin A (PPCA) has a serine carboxypeptidase activity of unknown physiological function. We now demonstrate that this protease activity triggers the degradation of the lysosome-associated membrane protein type 2a...