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Battenin

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Involved in microtubule-dependent, anterograde transport of late endosomes and lysosomes.

Below are the list of possible Battenin products. If you cannot find the target and/or product is not available in our catalog, please click here to contact us and request the product or submit your request for custom elisa kit production, custom recombinant protein production or custom antibody production. Custom ELISA Kits, Recombinant Proteins and Antibodies can be designed, manufactured and produced according to the researcher's specifications.
 

Battenin

 Battenin ELISA Kit
 Battenin Recombinant
 Battenin Antibody
Also known as Battenin (Batten disease protein) (Protein CLN3).
CLN3: Involved in microtubule-dependent, anterograde transport of late endosomes and lysosomes. Defects in CLN3 are the cause of neuronal ceroid lipofuscinosis type 3 (CLN3); also known as Batten disease. A form of neuronal ceroid lipofuscinosis. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage di
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seases characterized by intracellular accumulation of autofluorescent liposomal material, and clinically by seizures, dementia, visual loss, and/or cerebral atrophy. The hallmark of CLN3 is the ultrastructural pattern of lipopigment with a fingerprint profile, which can have 3 different appearances: pure within a lysosomal residual body; in conjunction with curvilinear or rectilinear profiles; and as a small component within large membrane-bound lysosomal vacuoles. The combination of fingerprint profiles within lysosomal vacuoles is a regular feature of blood lymphocytes from patients with CLN3. Belongs to the battenin family. 5 isoforms of the human protein are produced by alternative splicing.

Protein type: Apoptosis; Autophagy; Chaperone; Membrane protein, integral; Membrane protein, multi-pass; Mitochondrial; Vesicle

Chromosomal Location of Human Ortholog: 16p12.1

Cellular Component: autophagic vacuole; caveola; cytoplasm; early endosome; endoplasmic reticulum; Golgi apparatus; Golgi membrane; Golgi stack; integral to endoplasmic reticulum membrane; integral to membrane; late endosome; lipid raft; lysosomal membrane; lysosome; mitochondrion; neuron projection; nucleus; plasma membrane; synaptic vesicle; trans-Golgi network

Molecular Function: protein binding; unfolded protein binding

Biological Process: amino acid metabolic process; amyloid precursor protein catabolic process; arginine transport; associative learning; ceramide metabolic process; cytosolic calcium ion homeostasis; galactosylceramide metabolic process; globoside metabolic process; glucosylceramide metabolic process; ionotropic glutamate receptor signaling pathway; lysosomal lumen acidification; lysosome organization and biogenesis; negative regulation of apoptosis; negative regulation of catalytic activity; negative regulation of macroautophagy; negative regulation of neuron apoptosis; negative regulation of proteolysis; neuromuscular process controlling balance; neurotransmitter metabolic process; protein processing; receptor-mediated endocytosis; regulation of action potential; sphingomyelin metabolic process; vacuolar transport; vesicle transport along microtubule

Disease: Ceroid Lipofuscinosis, Neuronal, 3
 CLN3 ELISA Kit
 CLN3 Recombinant
 CLN3 Antibody
 BTS ELISA Kit
 BTS Recombinant
 BTS Antibody
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