Induced pluripotent stem cells from individuals with recessive dystrophic epidermolysis bullosa.
AuthorsTolar J, Xia L, Riddle MJ, Lees CJ, Eide CR, McElmurry RT, Titeux M, Osborn MJ, Lund TC, Hovnanian A, Wagner JE, Blazar BR
JournalJ Invest Dermatol
PubMed ID21124339
'Recessive dystrophic epidermolysis bullosa (RDEB) is an inherited blistering skin disorder caused by mutations in the COL7A1 gene-encoding type VII collagen (Col7), the major component of anchoring fibrils at the dermal-epidermal junction. Individuals with RDEB develop painful blisters and mucosal erosions, and currently, there are no effective forms of therapy. ... More
HBx genotype D represses GSTP1 expression and increases the oxidative level and apoptosis in HepG2 cells.
AuthorsNiu D, Zhang J, Ren Y, Feng H, Chen WN
JournalMol Oncol
PubMed ID19383368
'Epigenetics has been implicated in human cancer development. Epigenetic factors include HBx protein, which is able to induce hypermethylation and suppresses tumor suppressor genes. One of such tumor suppressor genes, GSTP1, shows reduced expression in many human cancers. Hypermethylation of GSTP1 is the most studied mechanism of its silence. In ... More
Hematopoietic differentiation of induced pluripotent stem cells from patients with mucopolysaccharidosis type I (Hurler syndrome).
AuthorsTolar J, Park IH, Xia L, Lees CJ, Peacock B, Webber B, McElmurry RT, Eide CR, Orchard PJ, Kyba M, Osborn MJ, Lund TC, Wagner JE, Daley GQ, Blazar BR
JournalBlood
PubMed ID21037085
Mucopolysaccharidosis type I (MPS IH; Hurler syndrome) is a congenital deficiency of a-L-iduronidase, leading to lysosomal storage of glycosaminoglycans that is ultimately fatal following an insidious onset after birth. Hematopoietic cell transplantation (HCT) is a life-saving measure in MPS IH. However, because a suitable hematopoietic donor is not found for ... More
Pathways for ATP release by bovine ciliary epithelial cells, the initial step in purinergic regulation of aqueous humor inflow.
AuthorsLi A, Leung CT, Peterson-Yantorno K, Mitchell CH, Civan MM
JournalAm J Physiol Cell Physiol
PubMed ID20926783
ATP release by nonpigmented (NPE) and pigmented (PE) ciliary epithelial cells is the enabling step in purinergic regulation of aqueous humor formation, but the release pathways are unknown. We measured ATP release from primary cultures of bovine mixed NPE and PE (bCE) cells and transformed bovine NPE and PE cells, ... More