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Primary and Secondary Elastin-Binding Protein Defect Leads to Impaired Elastogenesis in Fibroblasts from G(M1)-Gangliosidosis Patients
G(M1)-gangliosidosis is a lysosomal storage disorder caused by acid β-galactosidase deficiency. Aside from the lysosomal β-galactosidase enzyme, the β-galactosidase gene also encodes the elastin-binding protein (EBP), deficiency in which impairs elastogenesis. Using expression studies and Western bl...
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| Main Authors: | , , , , , , , |
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| Formato: | Artigo |
| Idioma: | Inglês |
| Publicado em: |
American Society for Investigative Pathology
2005
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| Assuntos: | |
| Acesso em linha: | https://ncbi.nlm.nih.gov/pmc/articles/PMC1613190/ https://ncbi.nlm.nih.gov/pubmed/16314480 |
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