Werner s syndrome

Li B, Navarro S, Kasahara N, Comai L (2004) Identification and biochemical characterization of a Werner s syndrome protein complex with Ku70/80 and poly(ADP-ribose) polymerase-1. J Biol Chem 279 13659-13667... 

Continuous intraperitoneal insulin infusion with implantable pumps has been assessed in 34 patients with poorly controlled diabetes (231). In two patients, the pump was explanted in one patient with Werner s syndrome (no subcutaneous fat) the pump was explanted because of infection in the pocket, and one pump was explanted because the patient had local complaints and psychological problems. One patient refused to be included. Patients were followed for 58 months. HbAic fell from 10.0 to 9.0% in the first year and remained there. Median days in hospital fell from 45 to 13 after 1 year. The quality of life was relatively low and many had psychiatric problems. Although long-term glycemic control improved and lengths of hospital stay were reduced, normal glucose control and normal quality of life could not be achieved. 

Goto, M., Werner s syndrome from clinics to genetics, Clin. Exp. Rheumatol., 18, 760-766, 2000. 

Kieras, F.J. et al., Elevation of urinary hyaluronic acid in Werner s syndrome and progeria, Biochem. Med. Metabol. Biol., 36, 276, 1986. 

A graphic overview of aging is seen in both early-onset (Hutchinson-Guillford syndrome) and adult-onset (Werner s syndrome) progeria, as well as in Down s syndrome. Other, less common forms of progeria have also been described (Cocayne syndrome, Wiedemann-Rautenstrauch syndrome, etc.). These disorders are all characterized by accelerated aging and a significantly reduced life span. 

G12. Goto, M., Rubenstein, M., Weber, J., Woods, K., and Drayna, D., Genetic linkage of Werner s syndrome to five markers of chromosome 8. Nature 355, 735-738 (1992). 

M7. Marklund, S, Nordensson, I, and Back, O., Normal CuZn superoxide dismutase, Mn superoxide dismutase, catalase and glutathione peroxidase in Werner s syndrome. J. Gerontol. 36 405 109 (1981). 

Suzuki N, Shimamoto A, Imamura O, Kuromitsu J, Kitao S, et al. 1997. DNA helicase activity in Werner s syndrome gene product synthesized in a baculovirus system. Nucleic Acids Res. 25 2973-78... 

Li J-L, Harrison JH, Reszka AP, Brosh RM Jr, Bohr NA, et al. 2001. Inhibition of the Bloom s and Werner s syndrome helicases by G-quadruplex interacting ligands. Biochemistry 40 15194-202... 

Gil. Goldstein, S., and Moerman, E. J., Heat-labile enzymes in Werner s syndrome fibroblasts. Nature (London) 255, 159 (1975). 

N9. Nordenson, I., Chromosome breaks in Werner s syndrome and their prevention in vitro. Hereditas 87, 151-154 (1977). 

Most people suffering from Werner s syndrome appear to have a... 

The RecQ helicases comprise a small, conserved enzyme family which is essential for maintenance of genomic stability in organisms from E. coli to human. " There is only one RecQ family member in E. coli (RecQ), one in S. cerevisiae (Sgsl), one in S. pombe (Rqhl), and five in Homo sapiens. Three human members of the RecQ family are associated with genomic instability and predisposition to malignancies WRN with Werner s syndrome, BLM with Bloom s syndrome, and RTS (or RECQ4) with Rothmund-Thompson syndrome. RecQ helicases are unusual in their ability to unwind G4 DNA, " ... 

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