{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T13:15:39Z","timestamp":1760188539498},"reference-count":18,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[1987,11,2]],"date-time":"1987-11-02T00:00:00Z","timestamp":562809600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[1988,9]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>We determined glycogen concentration and phosphorylase \u2018a+b\u2019 and phosphorylase a activities in platelets, mononuclear and polymorphonuclear cells from control subjects and patients with phosphorylase kinase deficiency (glycogen storage disease IX) and liver phosphorylase deficiency (glycogen storage disease VI). Variations according to cellular type and to subjects' age (1\u201340 years) were established. Variable glycogen overloading was found in all our patients. Glycogen storage disease (GSD) VI was characterized by a diminished total phosphorylase activity with a low or normal a\/(a+b) ratio of phosphorylase activity. GSD IX was characterized by a very low residual activity of phosphorylase a with an \u2018a+b\u2019 activity low or normal.<\/jats:p>","DOI":"10.1007\/bf01800366","type":"journal-article","created":{"date-parts":[[2005,6,21]],"date-time":"2005-06-21T17:04:24Z","timestamp":1119373464000},"page":"253-260","source":"Crossref","is-referenced-by-count":10,"title":["Use of platelets, mononuclear and polymorphonuclear cells in the diagnosis of glycogen storage disease type VI"],"prefix":"10.1002","volume":"11","author":[{"given":"N.","family":"Dahan","sequence":"first","affiliation":[{"name":"Laboratoire de Biochimie Appliqu\u00e9e, UER d'Hygi\u00e8ne et Protection de l'Homme et de son Environnement Centre pharmaceutique de Ch\u00e2tenay\u2010Malabry  78 rue du General Leclerc Le Kremlin\u2010Bic\u00eatre 94275 France"},{"name":"Laboratoire Central de Biochimie Centre hospitalier de Bic\u00eatre  78 rue du General Leclerc Le Kremlin\u2010Bic\u00eatre 94275 France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C.","family":"Baussan","sequence":"additional","affiliation":[{"name":"Laboratoire de Biochimie Appliqu\u00e9e, UER d'Hygi\u00e8ne et Protection de l'Homme et de son Environnement Centre pharmaceutique de Ch\u00e2tenay\u2010Malabry  78 rue du General Leclerc Le Kremlin\u2010Bic\u00eatre 94275 France"},{"name":"Laboratoire Central de Biochimie Centre hospitalier de Bic\u00eatre  78 rue du General Leclerc Le Kremlin\u2010Bic\u00eatre 94275 France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"N.","family":"Moatti","sequence":"additional","affiliation":[{"name":"Laboratoire de Biochimie Appliqu\u00e9e, UER d'Hygi\u00e8ne et Protection de l'Homme et de son Environnement Centre pharmaceutique de Ch\u00e2tenay\u2010Malabry  78 rue du General Leclerc Le Kremlin\u2010Bic\u00eatre 94275 France"},{"name":"Laboratoire Central de Biochimie Centre hospitalier de Bic\u00eatre  78 rue du General Leclerc Le Kremlin\u2010Bic\u00eatre 94275 France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Lemonnier","sequence":"additional","affiliation":[{"name":"Laboratoire de Biochimie Appliqu\u00e9e, UER d'Hygi\u00e8ne et Protection de l'Homme et de son Environnement Centre pharmaceutique de Ch\u00e2tenay\u2010Malabry  78 rue du General Leclerc Le Kremlin\u2010Bic\u00eatre 94275 France"},{"name":"Laboratoire Central de Biochimie Centre hospitalier de Bic\u00eatre  78 rue du General Leclerc Le Kremlin\u2010Bic\u00eatre 94275 France"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[1987,11,2]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1542\/peds.67.1.107","article-title":"Liver glycogenosis caused by a defective phosphorylase system. Haemolysate analysis","volume":"67","author":"Baussan C.","year":"1981","journal-title":"Pediatrics"},{"key":"e_1_2_1_3_1","first-page":"740","article-title":"Liver glycogen phosphorylase deficiency","volume":"102","author":"Drummond G. I.","year":"1970","journal-title":"Can. Med. Assoc. J."},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/0020-711X(78)90099-X"},{"key":"e_1_2_1_5_1","first-page":"1043","article-title":"H\u00e9t\u00e9rog\u00e9n\u00e9it\u00e9 de la glycog\u00e9nose type VI. Etude de l'activit\u00e9 de la phosphorylase leucocytaire dans deux familles","volume":"29","author":"Guibaud P.","year":"1972","journal-title":"Arch. Fr. 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