CLN3 blocking peptide
CLN3 Blocking Peptide (Center)
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a protein that is involved in lysosomal function. Mutations in this, as well as other neuronal ceroid-lipofuscinosis (CLN) genes, cause neurodegenerative diseases commonly known as Batten disease or collectively known as neuronal ceroid lipofuscinoses (NCLs). Many alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Jul 2008]
Uniprot Description
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 diseases 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: Vesicle; Membrane protein, integral; Autophagy; Apoptosis; Membrane protein, multi-pass; Chaperone; Mitochondrial
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
Research Articles on CLN3
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Product Notes
The CLN3 cln3 (Catalog #AAA9229476) is a Blocking Peptide and is intended for research purposes only. The product is available for immediate purchase. It is sometimes possible for the material contained within the vial of "CLN3, Blocking Peptide" 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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