Guinea Pig Ceroid-lipofuscinosis neuronal protein 6 (CLN6) ELISA Kit | CLN6 elisa kit
Guinea pig Ceroid-lipofuscinosis neuronal protein 6 (CLN6) ELISA Kit
NCBI and Uniprot Product Information
NCBI Description
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]
Uniprot Description
CLN6: Defects in CLN6 are the cause of neuronal ceroid lipofuscinosis type 6 (CLN6). A form of neuronal ceroid lipofuscinosis. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by intracellular accumulation of autofluorescent liposomal material, and clinically by seizures, dementia, visual loss, and/or cerebral atrophy. The lipopigment patterns observed most often in neuronal ceroid lipofuscinosis type 6 comprise mixed combinations of granular, curvilinear, and fingerprint profiles. Defects in CLN6 are the cause of neuronal ceroid lipofuscinosis type 4A (CLN4A). An adult-onset neuronal ceroid lipofuscinosis. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by intracellular accumulation of autofluorescent liposomal material, and clinically by seizures, dementia, visual loss, and/or cerebral atrophy. CLN4A has no visual involvement and is characterized by progressive myoclonic epilepsy.
Protein type: Membrane protein, integral; Membrane protein, multi-pass
Chromosomal Location of Human Ortholog: 15q23
Cellular Component: nucleoplasm; endoplasmic reticulum membrane; nuclear membrane; intracellular membrane-bound organelle; membrane; endoplasmic reticulum; endoplasmic reticulum lumen; integral to membrane
Molecular Function: protein binding; protein homodimerization activity
Biological Process: cholesterol metabolic process; visual perception; glycosaminoglycan metabolic process; positive regulation of proteolysis; lysosomal lumen acidification; protein catabolic process; locomotion during locomotory behavior; cellular macromolecule catabolic process; ganglioside metabolic process
Disease: Ceroid Lipofuscinosis, Neuronal, 6; Ceroid Lipofuscinosis, Neuronal, 4a, Autosomal Recessive
Research Articles on CLN6
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Product Notes
The Guinea Pig CLN6 cln6 (Catalog #AAA7240089) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA7240089 ELISA Kit recognizes Guinea Pig CLN6. It is sometimes possible for the material contained within the vial of "Ceroid-lipofuscinosis neuronal protein 6 (CLN6), ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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