Werner's syndrome as “Hyaluronuria”

Tokunaga, Masayuki; Futami, Tatsuhiko; Wakamatsu, Eikichi; Endo, Masahiko; Yosizawa, Zensaku

doi:10.1016/0009-8981(75)90283-1

Cited by 55 publications

(24 citation statements)

References 13 publications

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“…WS has been formerly inadvertently classified as a new group of hereditary mucopolysaccharidosis (44)(45)(46). Hyaluronan elevation either from urine and serum has been believed as a biomarker of normal aging and progeroid syndromes such as WS and progeria (46)(47)(48)(49) and the International Registry of Werner syndrome included hyaluronuria for diagnosing WS (http://www.…”

Section: Brief History Of Clinical Characterization Of Werner Syndromementioning

confidence: 99%

Werner syndrome: A changing pattern of clinical manifestations in Japan (1917-2008)

et al. 2013

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Section: Brief History Of Clinical Characterization Of Werner Syndromementioning

confidence: 99%

Werner syndrome: A changing pattern of clinical manifestations in Japan (1917-2008)

et al. 2013

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“…There have been found a striking shortening of the life-span and the slow elongation rate of the DNA chains of cultured fibroblasts (4,5), the increased proportion of heat labile enzymes (6,7), as well as the excessive excretion of urinary hyaluronic acid (8,9), all of which are probably related to the pathogenesis of this peculiar disorder. No overt immunological abnormalities have so far been reported in WS except a slight decrease in the proportion of T cells in the peripheral blood (3).…”

Section: Introductionmentioning

confidence: 99%

Immunological abnormalities of aging: an analysis of T lymphocyte subpopulations of Werner's syndrome.

Goto¹,

Horiuchi²,

Okumura³

et al. 1979

J. Clin. Invest.

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A B S T R A C T Two T lymphocyte-specific antisera, i.e. naturally-occurring auto-antibody to T cells of systemic lupus erythematosus patients (natural T cell toxic autoantibody) and heterologous antiserum against human brain tissue (antibrain-associated T-cell antigen), were used to detect cell surface antigens ofhuman peripheral T lymphocytes.Nylon column-purified T cells from normal aged individuals and patients with Werner's syndrome (a premature aging syndrome) were reacted with these auto-and heterologous antibodies followed by staining with appropriate fluorescence reagents. The cells were subjected to the automated analysis with fluorescence-activated cell sorter. Fluorescence profiles to T cells ofboth aged individuals ofover 90 yr and Werner's syndrome showed a very similar pattern, with a drastic decrease in the population that had high fluorescence intensity stained with either antiserum accompanied by the relative increase in the cell population that had low fluorescence intensity. Natural T cell toxic autoantibody comparable to that detected in systemic lupus erythematosus patients was found in the serum of six out of seven patients with Wemer's syndrome, whereas normal aged individuals produced no such an autoantibody. The results suggest that Wemer's syndrome has a change in the lymphocyte population very similar to old individuals, and that such a change is caused by the production of autoantibodies reactive to T lymphocytes.

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“…Urine was collected separately from 50 cases of orthopedic patients without any dietary restriction. Collection of 24-hr urine from each patient was carried out as described previously (Tokunaga et al 1975). …”

Section: Methodsmentioning

confidence: 99%

“…In our previous papers, we reported abnormal excretion of the following GAG in several connective tissue diseases : hyaluronic acid in Werner syndrome (Tokunaga et al 1975), epidermolysis bullosa dystrophica (recessive type) (Igarashi et al 1978) and progeria (Hutchinson-Gilford syndrome) (Tokunaga et al 1978a); dermatan sulfate in epidermolysis bullosa dystrophica et albopapuloidia (Pasini) (Endo et al 1974); partially degraded heparan sulfate and chondroitin sulfate in Rothmund Thomson syndrome (Tokunaga et al 1978b). In addition, Murata et al (1970Murata et al ( , 1973 reported increased excretion of chondroitin sulfate isomers and heparan sulfate in the urines of the patients with some systematic connective tissue diseases and Weber Christian disease, respectively.…”

mentioning

confidence: 97%