CLN6

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£544.00

SKU:: 311280
Reinheit:: Purified by immunoaffinity chromatography.
Lagerbedingungen:: -20°C
Hersteller:: United States Biological
Host:: Rabbit
Immunogen:: Synthetic peptide corresponding to internal of human CLN6.
Format:: Supplied as a liquid in PBS (without Mg2+ and Ca2+), pH 7.4, 0.02% sodium azide, 50% glycerol.
Weitere Details:: This gene is one of eight which have been associated with neuronal ceroid lipofuscinoses (NCL). Also referred to as Batten disease, NCL comprises a class of autosomal recessive, neurodegenerative disorders affecting children. The genes responsible likely encode proteins involved in the degradation of post-translationally modified proteins in lysosomes. The primary defect in NCL disorders is thought to be associated with lysosomal storage function.Ota T., Nat. Genet. 36:40-45(2004). The MGC Project Team; Genome Res. 14:2121-2127(2004). Mole S.E., Exp. Cell Res. 298:399-406(2004). Applications: Suitable for use in Western Blot and Immunohistochemistry. Other applications not tested. Recommended Dilution: Western Blot: 1:500-1:3000 Immunohistochemistry: 1:50-1:100 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 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.