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CLN6 Polyclonal Antibody, 100μg, (ATB-T0977)
Background: 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. [provided by RefSeq, Oct 2008]Product datasheet: Overview Product Description CLN6 Polyclonal Antibody, 100μg, (ATB-T0977) Image Species Reactivities Human Immunogen Synthesized peptide derived from the C-terminal region of human CLN6. Properties Form Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. Storage Instructions-20°C/1 year References: Deregulation of subcellular biometal homeostasis through loss of the metal transporter, Zip7, in a childhood neurodegenerative disorder. Grubman A, et al. Acta Neuropathol Commun, 2014 Feb 28. PMID 24581221 Free PMC Article CLN6 p.I154del mutation causing late infantile neuronal ceroid lipofuscinosis in a large consanguineous Moroccan family. Bouhouche A, et al. Indian J Pediatr, 2013 Aug. PMID 23180398 Mutation of the CLN6 gene in teenage-onset progressive myoclonus epilepsy. Andrade DM, et al. Pediatr Neurol, 2012 Sep. PMID 22883287 Kufs disease, the major adult form of neuronal ceroid lipofuscinosis, caused by mutations in CLN6. Arsov T, et al. Am J Hum Genet, 2011 May 13. PMID 21549341 Free PMC Article Pathogenic mutations cause rapid degradation of lysosomal storage disease-related membrane protein CLN6. Kurze AK, et al. Hum Mutat, 2010 Feb. PMID 20020536
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IntelixBio has swiftly become a household name in the medical industry, primarily owing to its cutting-edge medical lab equipment. Our commitment to excellence resonates through every facet of our products, ensuring that laboratories worldwide receive top-tier solutions for their diagnostic needs. Our medical lab equipment is engineered with precision and innovation, embodying the pinnacle of technological advancement in the field. From state-of-the-art analyzers to automated specimen-handling systems, our extensive catalog caters to diverse laboratory requirements, fostering efficiency and accuracy in every diagnostic process. Laboratories entrust IntelixBio's medical lab equipment for its unwavering reliability, empowering healthcare professionals with the tools they need to deliver optimal patient care.
At IntelixBio, our dedication to advancing healthcare is exemplified through our unparalleled range of medical lab equipment. Each product is meticulously crafted to meet the exacting standards of modern laboratory environments, facilitating seamless integration and operation. Our commitment to quality permeates every aspect of our offerings, ensuring that laboratories can consistently rely on our medical lab equipment to deliver precise and dependable results. With a comprehensive selection that spans from basic laboratory essentials to sophisticated diagnostic instruments, IntelixBio remains the foremost choice for institutions seeking excellence in medical equipment solutions. Trust IntelixBio to elevate your laboratory capabilities with our innovative medical lab equipment, empowering you to drive progress in healthcare delivery.
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At IntelixBio LLC, we offer high-quality medical lab equipment. Our lab supplies include:
- Protective gear
- Antibody immunodetection supplies
- Bioreagents
- Lab Supplies
- Immunohistochemistry Tools
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