Impaired neural differentiation of MPS IIIA patient induced pluripotent stem cell-derived neural progenitor cells

Autor: Jennifer T. Saville, Ainslie L. K. Derrick-Roberts, Brett V. Johnson, Megan S. Lord, Maria Fuller, Ha Na Kim, Lachlan A. Jolly, Rebecca J. Lehmann, Sharon Byers

Publikováno v: Molecular Genetics and Metabolism Reports, Vol 29, Iss, Pp 100811-(2021)
Molecular Genetics and Metabolism Reports

Mucopolysaccharidosis type IIIA (MPS IIIA) is characterised by a progressive neurological decline leading to early death. It is caused by bi-allelic loss-of-function mutations in SGSH encoding sulphamidase, a lysosomal enzyme required for heparan sul

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::8319b125d94dfde1df85ea9122f3ab4d
http://www.sciencedirect.com/science/article/pii/S2214426921001063

Preferential sites for intramolecular glucosepane cross-link formation in type I collagen: A thermodynamic study

Autor: Helen L. Birch, Nora H. de Leeuw, Thomas A. Collier, Anthony Nash

Publikováno v: Matrix Biology

The extracellular matrix (ECM) undergoes progressive age-related stiffening and loss of proteolytic digestibility due to an increase in concentration of advanced glycation end products (AGEs). The most abundant AGE, glucosepane, accumulates in collag

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::4085b2fc84fb49865a5522a39481a0ff

Tinospora cordifoliaconsumption ameliorates changes in kidney chondroitin sulphate/dermatan sulphate in diabetic rats

Autor: Darukeshwara Joladarashi, Nandini D. Chilkunda, Paramahans V. Salimath

Publikováno v: Journal of Nutritional Science

Diabetes is known to alter kidney extracellular matrix (ECM) components. Chondroitin sulphate (CS)/dermatan sulphate (DS), an ECM component, which plays an essential role in kidney is altered during diabetes. The focus of this study has been to exami

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::60ecd7f83cebb7522fbc44d576305fc7
https://doi.org/10.1017/jns.2012.6

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Impaired neural differentiation of MPS IIIA patient induced pluripotent stem cell-derived neural progenitor cells.

Autor: Lehmann RJ; Genetics and Molecular Pathology, SA Pathology (at the Women's and Children's Hospital), 72 King William Rd, North Adelaide, SA 5006, Australia.; Department of Molecular and Cellular Biology, University of Adelaide, Adelaide, SA 5005, Australia., Jolly LA; Adelaide Medical School, University of Adelaide, Adelaide, SA 5005, Australia.; Robinson Research Institute, University of Adelaide, Adelaide, SA 5005, Australia., Johnson BV; Adelaide Medical School, University of Adelaide, Adelaide, SA 5005, Australia.; Robinson Research Institute, University of Adelaide, Adelaide, SA 5005, Australia., Lord MS; Graduate School of Biomedical Engineering, UNSW, Sydney, NSW 2052, Australia., Kim HN; Graduate School of Biomedical Engineering, UNSW, Sydney, NSW 2052, Australia., Saville JT; Genetics and Molecular Pathology, SA Pathology (at the Women's and Children's Hospital), 72 King William Rd, North Adelaide, SA 5006, Australia., Fuller M; Genetics and Molecular Pathology, SA Pathology (at the Women's and Children's Hospital), 72 King William Rd, North Adelaide, SA 5006, Australia.; Adelaide Medical School, University of Adelaide, Adelaide, SA 5005, Australia.; Robinson Research Institute, University of Adelaide, Adelaide, SA 5005, Australia., Byers S; Genetics and Molecular Pathology, SA Pathology (at the Women's and Children's Hospital), 72 King William Rd, North Adelaide, SA 5006, Australia.; Department of Molecular and Cellular Biology, University of Adelaide, Adelaide, SA 5005, Australia.; Adelaide Medical School, University of Adelaide, Adelaide, SA 5005, Australia., Derrick-Roberts ALK; Genetics and Molecular Pathology, SA Pathology (at the Women's and Children's Hospital), 72 King William Rd, North Adelaide, SA 5006, Australia.; Adelaide Medical School, University of Adelaide, Adelaide, SA 5005, Australia.

Publikováno v: Molecular genetics and metabolism reports [Mol Genet Metab Rep] 2021 Oct 19; Vol. 29, pp. 100811. Date of Electronic Publication: 2021 Oct 19 (Print Publication: 2021).