Reactome | glycogen phosphorylase (PYGL) dimer b + 2 ATP => glycogen phosphorylase (PYGL) dimer a + 2 ADP

glycogen phosphorylase (PYGL) dimer b + 2 ATP => glycogen phosphorylase (PYGL) dimer a + 2 ADP

Stable Identifier

R-HSA-71588

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol

ReviewStatus

5/5

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glycogen phosphorylase (PYGL) dimer b + 2 ATP => glycogen phosphorylase (PYGL) dimer a + 2 ADP

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The cytosolic phosphorylase kinase complex catalyzes the phosphorylation of glycogen phosphorylase (PYGL). Two forms of phosphorylase kinase complex have been described (Brushia and Walsh 1999). The one annotated here, consisting of four copies each of PHKA2 (alpha regulatory) (van den Berg et al. 1995), PHKB (beta regulatory) (Burwinkel et al. 2003a), PHKG2 (gamma catalytic) (Burwinkel et al. 2003b; Maichele et al. 2006) and CALM (calmodulin) subunits is abundant in liver and its action on the form of phosphorylase (PYGL) abundant in liver is described.

While initial studies of glycogen phosphorylase PGYM from rabbit muscle suggested that it is a homotetramer (Keller and Cori 1953), more recent work indicates that under physiological conditions the enzyme occurs as a homodimer (Huang and Graves 1970) and a dimeric structure for human PYGL enzyme is inferred here.

Literature References

PubMed ID	Title	Journal	Year
13115432	Enzymic conversion of phosphorylase a to phosphorylase b Cori, GT, Keller, PJ	Biochim Biophys Acta	1953
8896567	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, Kilimann, MW, Maire, I, Sovik, O, Burwinkel, B	Nat Genet	1996
10487978	Phosphorylase kinase: the complexity of its regulation is reflected in the complexity of its structure Walsh, DA, Brushia, RJ	Front Biosci	1999
5461220	Correlation between subunit interactions and enzymatic activity of phosphorylase a. Method for determining equilibrium constants from initial rate measurements Huang, CY, Graves, DJ	Biochemistry	1970
12930917	Severe phenotype of phosphorylase kinase-deficient liver glycogenosis with mutations in the PHKG2 gene Chakraborty, PK, Kilimann, MW, Kvittingen, EA, Rootwelt, T, Burwinkel, B	Pediatr Res	2003
12825073	Muscle glycogenosis with low phosphorylase kinase activity: mutations in PHKA1, PHKG1 or six other candidate genes explain only a minority of cases Vorgerd, M, Kilimann, MW, Moses, SW, Schroers, A, Clemens, PR, Shin, YS, Burwinkel, B, Pongratz, D, Hu, B	Eur J Hum Genet	2003
7847371	X-linked liver phosphorylase kinase deficiency is associated with mutations in the human liver phosphorylase kinase alpha subunit. Berger, R, Smeitink, JA, Lamers, WH, van Amstel, HK, Malingré, HE, van den Berg, IE, van Beurden, EA, Poll-The, BT	Am J Hum Genet	1995