GSD6 FISH Probe
The GSD6 FISH probe is designed to hybridize to the GSD6 gene and is primarily used for detecting amplifications and deletions associated with the gene. This probe is FISH confirmed on normal peripheral blood metaphase spreads and interphase nuclei. The probe can be labeled in one of five colors. Each probe is sold in a 20 test kit (approximately 20 slides - 22x22 mm area) and includes hybridization buffer. Please note that due to design optimizations, prices are subject to change.
** This product is for in vitro and research use only. This product is not intended for diagnostic use.
| SKU | Test Kits | Buffer | Dye Color | Order Now |
|---|---|---|---|---|
| PYGL-20-OR (Standard Design) | 20 (40 μL) | 200 μL |  |
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| PYGL-20-RE | 20 (40 μL) | 200 μL |  |
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| PYGL-20-GO | 20 (40 μL) | 200 μL |  |
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| PYGL-20-GR | 20 (40 μL) | 200 μL |  |
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| PYGL-20-AQ | 20 (40 μL) | 200 μL |  |
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| PYGL-20-DIG | 20 (40 μL) | 200 μL |  |
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| PYGL-20-BIO | 20 (40 μL) | 200 μL |  |
Gene Summary
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.[provided by RefSeq, Feb 2011]
Gene Details
Gene Symbol: PYGL
Gene Name: Glycogen Phosphorylase L
Chromosome: CHR14: 51371934-51411248
Locus: 14q22.1
FISH Probe Protocols
| Protocol, Procedure, or Form Name | Last Modified | Download |
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Customer Publications
Product Details
Product: GSD6 FISH Probe
Test Kits: 20 (40 μL)
ISH Buffer: 200 μL
SKU: PYGL-20-OR
Material Safety Data Sheet: MSDS.pdf
Turnaround Time: 7-10 Business Days
Shipping Time: 1-2 Day Expedited Shipping