Anti-PYGL Antibody Picoband® fluoro594 Vervoeging

Glycogen phosphorylase, liver form (PYGL), also known as human liver glycogen phosphorylase (HLGP), is an enzyme that in humans is encoded by the PYGL gene on chromosome 14. This gene encodes a homodimeric protein that catalyses the cleavage of alpha-1,4-glucosidic bonds to release glucose-1-phosphate from liver glycogen stores. This protein switches from inactive phosphorylase B to active phosphorylase A by phosphorylation of serine residue 15. Activity of this enzyme is further regulated by multiple allosteric effectors and hormonal controls. Humans have three glycogen phosphorylase genes that encode distinct isozymes that are primarily expressed in liver, brain and muscle, respectively. The liver isozyme serves the glycemic demands of the body in general while the brain and muscle isozymes supply just those tissues. In glycogen storage disease type VI, also known as Hers disease, mutations in liver glycogen phosphorylase inhibit the conversion of glycogen to glucose and results in moderate hypoglycemia, mild ketosis, growth retardation and hepatomegaly. Alternative splicing results in multiple transcript variants encoding different isoforms.

ELAV-like protein 2; ELAV-like neuronal protein 1; Hu-antigen B; HuB; Nervous system-specific RNA-binding protein Hel-N1; ELAVL2; HUB

PYGL

5836

P06737

Human, Mouse, Rat

No cross-reactivity with other proteins.

E.coli-derived human PYGL recombinant protein (Position: K313-K804) .

Brain; neural-specific.

Flow Cytometry

DNA/RNA, Epigenetics and Nuclear Signaling, RNA Processing

Immunogen affinity purified.

Liquid

Binds RNA. Seems to recognize a GAAA motif. Can bind to its own 3'-UTR, the FOS 3'-UTR and the ID 3'-UTR.

1. Burwinkel, B., Bakker, H. D., Herschkovitz, E., Moses, S. W., Shin, Y. S., Kilimann, M. W. Mutations in the liver glycogen phosphorylase gene (PYGL) underlying glycogenosis type VI (Hers disease) . Am. J. Hum. Genet. 62: 785-791, 1998. 2. Chang, S., Rosenberg, M. J., Morton, H., Francomano, C. A., Biesecker, L. G. Identification of a mutation in liver glycogen phosphorylase in glycogen storage disease type VI. Hum. Molec. Genet. 7: 865-870, 1998. 3. Ercan-Fang, N., Gannon, M. C., Rath, V. L., Treadway, J. L., Taylor, M. R., Nuttall, F. Q. Integrated effects of multiple modulators on human liver glycogen phosphorylase alpha. Am. J. Physiol. Endocr. Metab. 283: E29-E37, 2002.

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

97 kDa

6

ELAV like RNA binding protein 2

Cytoplasm. Nucleus. Cytoplasm.

AP-2 complex subunit mu

Rabbit IgG

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.