The CLN8 Polyclonal Antibody (CAB16843) is a valuable tool for researchers studying CLN8, a protein associated with neuronal ceroid lipofuscinosis, a rare neurodegenerative disorder. This antibody, generated in rabbits, exhibits strong reactivity with human samples and has been validated for use in immunohistochemistry and ELISA applications. It specifically targets the CLN8 protein, allowing for accurate detection and analysis in various tissues and cell types, making it an essential reagent for investigations in neuroscience and rare genetic diseases.CLN8, a member of the CLN gene family, plays a crucial role in lysosomal function and lipid metabolism within neurons. Mutations in the CLN8 gene have been linked to neuronal ceroid lipofuscinosis, a group of disorders characterized by the buildup of lipofuscin in cells, leading to neurological decline.
Understanding the function of CLN8 is essential for uncovering the molecular mechanisms underlying these disorders and developing targeted therapies for affected individuals.The CLN8 Polyclonal Antibody offers researchers a reliable tool for studying the expression and localization of CLN8 in disease models and patient samples, providing valuable insights into the pathogenesis of neuronal ceroid lipofuscinosis and potential therapeutic strategies. Its high specificity and sensitivity make it an indispensable asset for advancing research in neurodegenerative diseases and lysosomal storage disorders.
Product Name:
CLN8 Rabbit Polyclonal Antibody
SKU:
CAB16843
Size:
20uL, 100uL
Isotype:
IgG
Host Species:
Rabbit
Reactivity:
Human
Immunogen:
A synthetic peptide corresponding to a sequence within amino acids 200-286 of human CLN8 (NP_061764.2).
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 a disorder characterized by progressive epilepsy with cognitive disabilities (EPMR), 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.
Purification Method:
Affinity purification
Gene ID:
2055
Storage Buffer:
Store at -20℃. Avoid freeze / thaw cycles.Buffer: PBS with 0.01% thimerosal,50% glycerol,pH7.3.
Western blot analysis of various lysates using CLN8 Rabbit pAb (CAB16843) at 1:1000 dilution.Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution.Lysates/proteins: 25μg per lane.Blocking buffer: 3% nonfat dry milk in TBST.Detection: ECL Enhanced Kit (AbGn00021).Exposure time: 90s.
