Hu H - Search Results

1

AAV-mediated gene therapy for galactosialidosis: A long-term safety and efficacy study.

Hu H, Mosca R, Gomero E, van de Vlekkert D, Campos Y, Fremuth LE, Brown SA, Weesner JA, Annunziata I, d'Azzo A. Hu H, et al. Mol Ther Methods Clin Dev. 2021 Oct 28;23:644-658. doi: 10.1016/j.omtm.2021.10.007. eCollection 2021 Dec 10. Mol Ther Methods Clin Dev. 2021. PMID: 34901309 Free PMC article.

2

Expression specificity of GFAP transgenes.

Su M, Hu H, Lee Y, d'Azzo A, Messing A, Brenner M. Su M, et al. Among authors: hu h. Neurochem Res. 2004 Nov;29(11):2075-93. doi: 10.1007/s11064-004-6881-1. Neurochem Res. 2004. PMID: 15662842

3

Neuraminidase 1 is a negative regulator of lysosomal exocytosis.

Yogalingam G, Bonten EJ, van de Vlekkert D, Hu H, Moshiach S, Connell SA, d'Azzo A. Yogalingam G, et al. Among authors: hu h. Dev Cell. 2008 Jul;15(1):74-86. doi: 10.1016/j.devcel.2008.05.005. Dev Cell. 2008. PMID: 18606142 Free PMC article.

4

Vacuolization and alterations of lysosomal membrane proteins in cochlear marginal cells contribute to hearing loss in neuraminidase 1-deficient mice.

Wu X, Steigelman KA, Bonten E, Hu H, He W, Ren T, Zuo J, d'Azzo A. Wu X, et al. Among authors: hu h. Biochim Biophys Acta. 2010 Feb;1802(2):259-68. doi: 10.1016/j.bbadis.2009.10.008. Epub 2009 Oct 24. Biochim Biophys Acta. 2010. PMID: 19857571 Free PMC article.

5

Muscle degeneration in neuraminidase 1-deficient mice results from infiltration of the muscle fibers by expanded connective tissue.

Zanoteli E, van de Vlekkert D, Bonten EJ, Hu H, Mann L, Gomero EM, Harris AJ, Ghersi G, d'Azzo A. Zanoteli E, et al. Among authors: hu h. Biochim Biophys Acta. 2010 Jul-Aug;1802(7-8):659-72. doi: 10.1016/j.bbadis.2010.04.002. Epub 2010 Apr 11. Biochim Biophys Acta. 2010. PMID: 20388541 Free PMC article.

6

Preclinical dose-finding study with a liver-tropic, recombinant AAV-2/8 vector in the mouse model of galactosialidosis.

Hu H, Gomero E, Bonten E, Gray JT, Allay J, Wu Y, Wu J, Calabrese C, Nienhuis A, d'Azzo A. Hu H, et al. Mol Ther. 2012 Feb;20(2):267-74. doi: 10.1038/mt.2011.227. Epub 2011 Oct 18. Mol Ther. 2012. PMID: 22008912 Free PMC article.

7

Chaperone-mediated gene therapy with recombinant AAV-PPCA in a new mouse model of type I sialidosis.

Bonten EJ, Yogalingam G, Hu H, Gomero E, van de Vlekkert D, d'Azzo A. Bonten EJ, et al. Among authors: hu h. Biochim Biophys Acta. 2013 Oct;1832(10):1784-92. doi: 10.1016/j.bbadis.2013.06.002. Epub 2013 Jun 12. Biochim Biophys Acta. 2013. PMID: 23770387 Free PMC article.

8

Lysosomal NEU1 deficiency affects amyloid precursor protein levels and amyloid-β secretion via deregulated lysosomal exocytosis.

Annunziata I, Patterson A, Helton D, Hu H, Moshiach S, Gomero E, Nixon R, d'Azzo A. Annunziata I, et al. Among authors: hu h. Nat Commun. 2013;4:2734. doi: 10.1038/ncomms3734. Nat Commun. 2013. PMID: 24225533 Free PMC article.

9

Excessive exosome release is the pathogenic pathway linking a lysosomal deficiency to generalized fibrosis.

van de Vlekkert D, Demmers J, Nguyen XX, Campos Y, Machado E, Annunziata I, Hu H, Gomero E, Qiu X, Bongiovanni A, Feghali-Bostwick CA, d'Azzo A. van de Vlekkert D, et al. Among authors: hu h. Sci Adv. 2019 Jul 17;5(7):eaav3270. doi: 10.1126/sciadv.aav3270. eCollection 2019 Jul. Sci Adv. 2019. PMID: 31328155 Free PMC article.

10

Galactosialidosis: preclinical enzyme replacement therapy in a mouse model of the disease, a proof of concept.

Cadaoas J, Hu H, Boyle G, Gomero E, Mosca R, Jayashankar K, Machado M, Cullen S, Guzman B, van de Vlekkert D, Annunziata I, Vellard M, Kakkis E, Koppaka V, d'Azzo A. Cadaoas J, et al. Among authors: hu h. Mol Ther Methods Clin Dev. 2020 Nov 20;20:191-203. doi: 10.1016/j.omtm.2020.11.012. eCollection 2021 Mar 12. Mol Ther Methods Clin Dev. 2020. PMID: 33426146 Free PMC article.

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