|Other Names||PDZ and LIM domain protein 3, Actinin-associated LIM protein, Alpha-actinin-2-associated LIM protein, PDLIM3, ALP|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||May play a role in the organization of actin filament arrays within muscle cells.|
|Cellular Location||Cytoplasm, myofibril, sarcomere, Z line. Note=Localizes to myofiber Z-lines|
|Tissue Location||Isoform 1 is highly expressed in differentiated skeletal muscle. Isoform 2 is heart-specific|
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The protein encoded by this gene contains a PDZ domain anda LIM domain, indicating that it may be involved in cytoskeletalassembly. In support of this, the encoded protein has been shown tobind the spectrin-like repeats of alpha-actinin-2 and to colocalizewith alpha-actinin-2 at the Z lines of skeletal muscle. This geneis found near a region of chromosome 4 that has been implicated infacioscapulohumeral muscular dystrophy, but this gene does notappear to be involved in the disease. Two transcript variantsencoding different isoforms have been found for this gene.
Hershberger, R.E., et al. Clin Transl Sci 1(1):21-26(2008)Keira, Y., et al. Neurosci. Res. 57(4):513-521(2007)Arola, A.M., et al. Mol. Genet. Metab. 90(4):435-440(2007)Klaavuniemi, T., et al. J. Biol. Chem. 279(25):26402-26410(2004)Bouju, S., et al. Neuromuscul. Disord. 9(1):3-10(1999)
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