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2. Liver glycogen storage diseases due to phosphorylase system deficiencies: diagnosis thanks to non invasive blood enzymatic and molecular studies. Davit-Spraul A; Piraud M; Dobbelaere D; Valayannopoulos V; Labrune P; Habes D; Bernard O; Jacquemin E; Baussan C Mol Genet Metab; 2011; 104(1-2):137-43. PubMed ID: 21646031 [TBL] [Abstract][Full Text] [Related]
3. Phosphorylase b kinase deficiency in a boy with glycogenosis affecting both liver and muscle. Madlom M; Besley GT; Cohen PT; Marrian VJ Eur J Pediatr; 1989 Oct; 149(1):52-3. PubMed ID: 2606129 [TBL] [Abstract][Full Text] [Related]
4. Localization of a new type of X-linked liver glycogenosis to the chromosomal region Xp22 containing the liver alpha-subunit of phosphorylase kinase (PHKA2). Hendrickx J; Coucke P; Hors-Cayla MC; Smit GP; Shin YS; Deutsch J; Smeitink J; Berger R; Lee P; Fernandes J Genomics; 1994 Jun; 21(3):620-5. PubMed ID: 7959740 [TBL] [Abstract][Full Text] [Related]
5. [Regulation of glycogen metabolism in the liver and hepatic glycogenosis due to phosphorylase system deficiency]. Lemonnier A; Moatti N; Baussan C C R Seances Soc Biol Fil; 1979; 173(2):483-95. PubMed ID: 228813 [TBL] [Abstract][Full Text] [Related]
7. [Genetic heterogeneity and the diagnosis of hepatic glycogenoses]. Lemonnier A; Baussan C; Moatti N C R Seances Soc Biol Fil; 1984; 178(4):327-47. PubMed ID: 6241011 [TBL] [Abstract][Full Text] [Related]
9. Variability of biochemical and clinical phenotype in X-linked liver glycogenosis with mutations in the phosphorylase kinase PHKA2 gene. Burwinkel B; Amat L; Gray RG; Matsuo N; Muroya K; Narisawa K; Sokol RJ; Vilaseca MA; Kilimann MW Hum Genet; 1998 Apr; 102(4):423-9. PubMed ID: 9600238 [TBL] [Abstract][Full Text] [Related]
10. Human muscle glycogenosis due to phosphorylase kinase deficiency associated with a nonsense mutation in the muscle isoform of the alpha subunit. Wehner M; Clemens PR; Engel AG; Kilimann MW Hum Mol Genet; 1994 Nov; 3(11):1983-7. PubMed ID: 7874115 [TBL] [Abstract][Full Text] [Related]
11. Mutations in the testis/liver isoform of the phosphorylase kinase gamma subunit (PHKG2) cause autosomal liver glycogenosis in the gsd rat and in humans. Maichele AJ; Burwinkel B; Maire I; Søvik O; Kilimann MW Nat Genet; 1996 Nov; 14(3):337-40. PubMed ID: 8896567 [TBL] [Abstract][Full Text] [Related]
12. Autosomal glycogenosis of liver and muscle due to phosphorylase kinase deficiency is caused by mutations in the phosphorylase kinase beta subunit (PHKB). Burwinkel B; Maichele AJ; Aagenaes O; Bakker HD; Lerner A; Shin YS; Strachan JA; Kilimann MW Hum Mol Genet; 1997 Jul; 6(7):1109-15. PubMed ID: 9215682 [TBL] [Abstract][Full Text] [Related]
13. Mapping of the gene for X-linked liver glycogenosis due to phosphorylase kinase deficiency to human chromosome region Xp22. Willems PJ; Hendrickx J; Van der Auwera BJ; Vits L; Raeymaekers P; Coucke PJ; Van den Bergh I; Berger R; Smit GP; Van Broeckhoven C Genomics; 1991 Apr; 9(4):565-9. PubMed ID: 1674721 [TBL] [Abstract][Full Text] [Related]
14. Glycogen phosphorylase and its converter enzymes in haemolysates of normal human subjects and of patients with type VI glycogen-storage disease. A study of phosphorylase kinase deficiency. Lederer B; Van Hoof F; Van den Berghe G; Hers H Biochem J; 1975 Apr; 147(1):23-35. PubMed ID: 168880 [TBL] [Abstract][Full Text] [Related]
15. Mutation hotspots in the PHKA2 gene in X-linked liver glycogenosis due to phosphorylase kinase deficiency with atypical activity in blood cells (XLG2). Burwinkel B; Shin YS; Bakker HD; Deutsch J; Lozano MJ; Maire I; Kilimann MW Hum Mol Genet; 1996 May; 5(5):653-8. PubMed ID: 8733134 [TBL] [Abstract][Full Text] [Related]
16. cDNA cloning of a liver isoform of the phosphorylase kinase alpha subunit and mapping of the gene to Xp22.2-p22.1, the region of human X-linked liver glycogenosis. Davidson JJ; Ozçelik T; Hamacher C; Willems PJ; Francke U; Kilimann MW Proc Natl Acad Sci U S A; 1992 Mar; 89(6):2096-100. PubMed ID: 1372435 [TBL] [Abstract][Full Text] [Related]
17. Fatal congenital heart glycogenosis caused by a recurrent activating R531Q mutation in the gamma 2-subunit of AMP-activated protein kinase (PRKAG2), not by phosphorylase kinase deficiency. Burwinkel B; Scott JW; Bührer C; van Landeghem FK; Cox GF; Wilson CJ; Grahame Hardie D; Kilimann MW Am J Hum Genet; 2005 Jun; 76(6):1034-49. PubMed ID: 15877279 [TBL] [Abstract][Full Text] [Related]