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G8169-96B1 Rabbit Anti-Glycogen Phosphorylase, Muscle Form, CT (PYGM, Myophosphorylase)

Specifications
References
Clone Type
Polyclonal
Host
Rabbit
Source
Human
Swiss Prot
P11217
Isotype
IgG
Grade
Purified
Applications
E IHC WB
Crossreactivity
Hu
Accession #
NP_005600
Shipping Temp
Blue Ice
Storage Temp
-20°C

PYGM catalyzes and regulates the breakdown of glycogen to glucose-1-phosphate. Defects in PYGM are the cause of glycogen storage disease type 5 (GSD5), also known as McArdle disease. GSD5 is a metabolic disorder resulting in myopathy characterized by exercise intolerance, cramps, muscle weakness and recurrent myoglobinuria.

Applications
Suitable for use in ELISA, Western Blot, and Immunohistochemistry. Other applications not tested.
Recommended Dilution
ELISA: 1:1,000 Western Blot: 1:100-1:500 Immunohistochemistry: 1:10-1:50 Optimal dilutions to be determined by the researcher.
Storage and Stability
May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Immunogen
Synthetic peptide selected from the C-terminal region of human PYGM (KLH).
Form
Supplied as a liquid in PBS, 0.09% sodium azide.
Purity
Purified by ammonium sulfate precipitation
Specificity
Recognizes human PYGM.
References
1. Tsoi, S.C., et al., J. Soc. Gynecol. Investig. 10(8):496-502 (2003). 2. Bruno, C., et al., Neuromuscul. Disord. 12(5):498-500 (2002). 3. Hadjigeorgiou, G.M., et al., Neuromuscul. Disord. 12(9):824-827 (2002). 4. Deschauer, M., et al., Mol. Genet. Metab. 74(4):489-491 (2001). 5. Kubisch, C., et al., Hum. Mutat. 12(1):27-32 (1998).
USBio References
No references available
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