034005
Clone Type
PolyclonalHost
RabbitSource
HumanIsotype
IgGGrade
Affinity PurifiedApplications
E WBCrossreactivity
HuAccession #
NP_061764.2Gene ID
2055Gene #
CLN8Shipping Temp
Blue IceStorage Temp
-20°CRabbit Anti-CLN8, CT (CLN8, C8orf61, Protein CLN8)
CLN8, C8orf61, Protein CLN8
This gene encodes a transmembrane protein belonging to a family of proteins containing TLC domains, which are postulated to function in lipid synthesis, transport, or sensing. The protein localizes to the endoplasmic reticulum (ER), and may recycle between the ER and ER-Golgi intermediate compartment. Mutations in this gene are associated with progressive epilepsy with mental retardation (EMPR), which is a subtype of neuronal ceroid lipofuscinoses (NCL). Patients with mutations in this gene have altered levels of sphingolipid and phospholipids in the brain.
Applications
Suitable for use in Western Blot, ELISA
Recommended Dilution
ELISA: 1:1,000 Western Blot: 1:100-500
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. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Immunogen
CLN8 antibody is generated from rabbits immunized with a KLH conjugated synthetic between 250-280 amino acids selected from the C-terminal region of human CLN8.
Form
Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.
Purity
Purified by Protein A affinity chromatography.
Intended for research use only. Not for use in human, therapeutic, or diagnostic applications.
References
Reinhardt, K., et al. Clin. Genet. 77(1):79-85(2010)|Vantaggiato, C., et al. Hum. Mutat. 30(7):1104-1116(2009)|Kousi, M., et al. Brain 132 (PT 3), 810-819 (2009) :|Striano, P., et al. Epilepsy Behav 10(1):187-191(2007)|Hermansson, M., et al. J. Neurochem. 95(3):609-617(2005)USBio References
No references available