GSD II

Kroos M, Hoogeveen-Westerveld M, Michelakakis H, Pomponio R, Van der Ploeg A, Halley D, Reuser A, , Augoustides-Savvopoulou P, Ausems M, Llona JB, Bautista Lorite J, van der Beek N, Bonafe L, Cuk M, D'Hooghe M, Engelen B, Farouk A, Fumic K, Garcia-Delgado E, Herzog A, Hurst J, Jones S, Kariminejad MH, Küçükçongar A, Lissens W, Lund A, Majoor-Krakauer D, Kumamoto S, Maravi E, Marie S, Mengel E, Mavridou I, Munteis Olivas E, Najmabadi H, Okumiya T, Peric S, Paschke E, Plecko B, Robberecht W, Serdaroglu P, Shboul M, Tansek MZ, Tarnutzer A, Stojanovic VR, Tylki-Szymanska A, Venâncio M, Verhoeven K. Update of the pompe disease mutation database with 60 novel GAA sequence variants and additional studies on the functional effect of 34 previously reported variants. Hum. Mutat. 2012 May 29;33(8):1161–5. doi: 10.1002/humu.22108.; Hermans MMP, de Graaff E, Kroos MA, Wisselaar HA, Oostra BA, Reuser AJJ. Identification of a point mutation in the human lysosomal a-glucosidase gene causing infantile glycogenosis type II. Biochemical and Biophysical Research Communications. 1991 Sep;179(2):919–26. doi: 10.1016/0006-291x(91)91906-s.; Zhong N, Martiniuk F, Tzall S, Hirschhorn R. Identification of a missense mutation in one allele of a patient with Pompe disease, and use of endonuclease digestion of PCR-amplified RNA to demonstrate lack of mRNA expression from the second allele. Am J Hum Genet. 1991 Sep;49(3):635–45. PMID: 1652892; PMCID: PMC1683123.; Brown BI, Brown DH, Jeffrey PL. Simultaneous absence of alpha-1,4-glucosidase and alpha-1,6-glucosidase activities (pH 4) in tissues of children with type II glycogen storage disease. Biochemistry. 1970 Mar 17;9(6):1423–8. doi: 10.1021/bi00808a017. PMID: 5264799.; HERS HG. alpha-Glucosidase deficiency in generalized glycogenstorage disease (Pompe's disease). Biochem J. 1963 Jan;86():11–6. PMID: 13954110; PMCID: PMC1201703.

Metabolites

H2O

Formula: H2O (18.0105642)

CAS ID: 7732-18-5

Manganese(2+)

Formula: Mn (54.938046)

CAS ID: 7439-96-5



Enzyme

EC Number name full name note
2.4.1.186 glycogenin glucosyltransferase UDP-alpha-D-glucose:glycogenin alpha-D-glucosyltransferase


Proteins

Protein ID name full name