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- Lorena Gallego-Villar; Belén Pérez; Magdalena Ugarte; Lourdes R. Desviat; Eva Richard
- Biochemical and biophysical research communications 2014 v.452 pp. 457-461
- acidosis; adjuvants; animal models; antioxidants; biotin; fibroblasts; glutathione peroxidase; humans; mitochondria; movement disorders; patients; propionyl-CoA carboxylase; resveratrol; superoxide dismutase; therapeutics
- ... Propionic acidemia (PA), caused by a deficiency of the mitochondrial biotin dependent enzyme propionyl-CoA carboxylase (PCC) is one of the most frequent organic acidurias in humans. Most PA patients present in the neonatal period with metabolic acidosis and hyperammonemia, developing different neurological symptoms, movement disorders and cardiac complications. There is strong evidence indicating ...
- Zhaoxiang Liu; Hua Zhang; Hong Xia; Baocheng Wang; Renwen Zhang; Qun Zeng; Lingzhi Guo; Kui Shen; BaTa Wang; Yanheng Zhong; Zhizhong Li; Guodong Sun
- Biochemical and biophysical research communications 2019 v.512 no.2 pp. 367-372
- CD8-positive T-lymphocytes; animal injuries; animal models; biotin; cerebrospinal fluid; cytokines; enzyme-linked immunosorbent assay; flow cytometry; humans; infectious diseases; inflammation; mice; permeability; spinal cord; staining
- ... Perforin plays an important role in autoimmune and infectious diseases, but its function in immune inflammatory responses after spinal cord injury (SCI) has received insufficient attention. The goal of this study is to determine the influence of perforin after spinal cord injury (SCI) on secondary inflammation. Compared recovery from SCI in perforin knockout (Prf1−/−) and wild-type(WT)mice, WT mic ...