Rabbit CLN3 Polyclonal Antibody | anti-CLN3 antibody

CLN3 Antibody (Center)

Cat. Num. AAA9211565

• Gene Names: CLN3; BTS; JNCL
• Reactivity: Human, mouse (Predicted Reactivity: Monkey)
• Applications: Flow Cytometry, Functional Assay, ELISA, Western Blot, Immunohistochemistry
• Purity: Purified Rabbit Polyclonal Antibody (Pab)
• Synonyms: CLN3; Polyclonal Antibody; CLN3 Antibody (Center); Battenin; Batten disease protein; Protein CLN3; BTS; anti-CLN3 antibody

• Host: Rabbit
• Reactivity: Human, mouse (Predicted Reactivity: Monkey)
• Clonality: Polyclonal
• Isotype: Rabbit Ig
• Specificity: This CLN3 antibody is generated from a rabbit immunized with a KLH conjugated synthetic peptide between 250-284 amino acids from the Central region of human CLN3.
• Purity/Purification: Purified Rabbit Polyclonal Antibody (Pab)
• Form/Format: Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.
• Sequence Length: 438

• Applicable Applications for anti-CLN3 antibody: Flow Cytometry (FC/FACS), ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)
• Application Notes: WB~~1:500
• Crown Antibody: Yes
• Antigen Source: HUMAN
• Function: Mediates microtubule-dependent,anterograde transport connecting the Golginetwork, endosomes, autophagosomes,lysosomes and plasma membrane, andparticipates in several cellular processes suchas regulation of lysosomal pH, lysosomeprotein degradation, receptor- mediatedendocytosis, autophagy, transport of proteinsand lipids from the TGN, apoptosis andsynaptic transmission (PubMed:10924275, PubMed:18817525,PubMed:18317235, PubMed:22261744,PubMed:15471887, PubMed:20850431).Facilitates the proteins transport fromtrans-Golgi network (TGN)-to othermembrane compartments such as transportof microdomain-associated proteins to theplasma membrane, IGF2R transport to thelysosome where it regulates the CTSD releaseleading to regulation of CTSD maturation andthereby APP intracellular processing(PubMed:10924275, PubMed:18817525).Moreover regulates CTSD activity in responseto osmotic stress (PubMed:23840424, PubMed:28390177). Alsobinds galactosylceramide and transports itfrom the trans Golgi to the rafts, which mayhave immediate and downstream effects oncell survival by modulating ceramidesynthesis (PubMed:18317235). At theplasma memebrane, regulatesactin-dependent events including filopodiaformation, cell migration, and pinocytosisthrough ARF1-CDC42 pathway and also thecytoskeleton organization through interactionwith MYH10 and fodrin leading to theregulation of the plasma membraneassociation of Na+, K+ ATPase complex(PubMed:20850431). Regulatessynaptic transmission in the amygdala,hippocampus, and cerebellum throughregulation of synaptic vesicles density andtheir proximity to active zones leading tomodulation of short-term plasticity andage-dependent anxious behavior, learningand memory (By similarity). Regulatesautophagic vacuoles (AVs) maturation bymodulating the trafficking between endocyticand autophagolysosomal/lysosomalCLN3 Antibody (Center) - Background Involved in microtubule-dependent,anterograde transport of late endosomes andlysosomes.CLN3 Antibody (Center) - References Lerner T.J.,et al.Cell 82:949-957(1995).Mitchison H.M.,et al.Genomics 40:346-350(1997).LaFauci G.,et al.Submitted (JUL-1997) to theEMBL/GenBank/DDBJ databases.LaFauci G.,et al.Submitted (JUL-1998) to theEMBL/GenBank/DDBJ databases.Ota T.,et al.Nat. Genet. 36:40-45(2004). compartments, which involves vesicle fusionleading to regulation of degradation process(By similarity). Participates also in cellularhomeostasis of compounds such as, water,ions, amino acids, proteins and lipids inseveral tissue namely in brain and kidneythrough regulation of their transport andsynthesis (PubMed:17482562).
• Cellular Location: Lysosome membrane; Multi-pass membraneprotein. Late endosome. Lysosome. Golgiapparatus. Golgi apparatus membrane. Golgiapparatus, Golgi stack. Golgi apparatus,trans-Golgi network. Cell membraneRecycling endosome. Membrane raft.Membrane, caveola. Early endosomemembrane. Cell junction, synapse,synaptosome{ECO:0000250|UniProtKB:Q61124}. Lateendosome membrane{ECO:0000250|UniProtKB:Q61124}.Cytoplasmic vesicle, autophagosome{ECO:0000250|UniProtKB:Q61124}.Note=CLN3 is not present in lateendosomes/lysosomes in fibroblasts andneurons (PubMed:15240864) Trafficks fromcell membrane to Golgi via endosomes(PubMed:15240864) Osmotic stress changesthe subcellular localization of CLN3(PubMed:23840424). Trafficks to intracellularcompartments via the plasma membranetthrough AP3M1-dependent mechanisms(PubMed:14644441) Excluded from thesynaptic vesicles (By similarity){ECO:0000250|UniProtKB:Q61124,ECO:0000269|PubMed:14644441,ECO:0000269|PubMed:15240864,ECO:0000269|PubMed:23840424}
• Tissue Location: Expressed in the cortical brain, pancreas,spleen, and testis with weaker expression inthe peripheral nerve (at protein level). Highlyexpressed in gray matter (at protein level)
• Preparation and Storage: Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.

Western Blot (WB)

(Anti-CLN3 Antibody (Center) at 1:1000 dilution + Neuro-2a whole cell lysate Lysates/proteins at 20 µg per lane. Secondary Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution. Predicted band size : 48 kDaBlocking/Dilution buffer: 5% NFDM/TBST.)

Western Blot (WB)

(All lanes : Anti-CLN3 Antibody (Center) at 1:500 dilution Lane 1: Mouse kidney lysate Lane 2: Neuro-2a whole cell lysate Lysates/proteins at 20 µg per lane. Secondary Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution. Predicted band size : 48 kDa Blocking/Dilution buffer: 5% NFDM/TBST.)

Related Product Information for anti-CLN3 antibody

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

Product Categories/Family for anti-CLN3 antibody

Crown Antibodies; Cell Biology; Neuroscience

References

Lerner T.J.,et al.Cell 82:949-957(1995).
Mitchison H.M.,et al.Genomics 40:346-350(1997).
LaFauci G.,et al.Submitted (JUL-1997) to the EMBL/GenBank/DDBJ databases.
LaFauci G.,et al.Submitted (JUL-1998) to the EMBL/GenBank/DDBJ databases.
Ota T.,et al.Nat. Genet. 36:40-45(2004).

NCBI and Uniprot Product Information

• NCBI GI #: 4502889
• NCBI GeneID: 1201
• NCBI Accession #: NP_000077.1
• NCBI GenBank Nucleotide #: NM_000086.2
• UniProt Accession #: Q13286; O00668; O95089; Q549S9; Q9UP09; Q9UP11; Q9UP12; Q9UP13; Q9UP14; B2R7J1; B4DXL3
• Molecular Weight: 47623
• NCBI Official Full Name: battenin isoform a
• NCBI Official Synonym Full Names: ceroid-lipofuscinosis, neuronal 3
• NCBI Official Symbol: CLN3
• NCBI Official Synonym Symbols: BTS; JNCL
• NCBI Protein Information: battenin
• UniProt Protein Name: Battenin
• Protein Family: Protein
• UniProt Gene Name: CLN3
• UniProt Synonym Gene Names: BTS
• UniProt Entry Name: CLN3_HUMAN

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

Chromosomal Location of Human Ortholog: 16p12.1

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

Molecular Function: protein binding; unfolded protein binding

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

Disease: Ceroid Lipofuscinosis, Neuronal, 3