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CLN3 Antibody (Center)

Purified Rabbit Polyclonal Antibody (Pab)

     
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  • WB - CLN3 Antibody (Center) AW5327-U100
    Western blot analysis of lysates from A431,KG-1 cell line,mouse kidney,mouse liver tissue lysate,SH-SY5Y cell line (from left to right), using CLN3 Antibody (Center)(Cat. #AW5327). AW5327 was diluted at 1:1000 at each lane. A goat anti-rabbit IgG H&L(HRP) at 1:10000 dilution was used as the secondary antibody.Lysates at 20ug per lane.
    detail
  • IHC-P - CLN3 Antibody (Center) AW5327-U100
    Immunohistochemical analysis of paraffin-embedded H. liver section using CLN3 Antibody (Center)(Cat#AW5327). AW5327 was diluted at 1:25 dilution. A undiluted biotinylated goat polyvalent antibody was used as the secondary, followed by DAB staining.
    detail
  • FC - CLN3 Antibody (Center) AW5327-U100
    Flow cytometric analysis of Hela cells using CLN3 Antibody (Center)(green, Cat#AW5327c) compared to an isotype control of rabbit IgG(blue). AW5327 was diluted at 1:25 dilution. An Alexa Fluor® 488 goat anti-rabbit lgG at 1:400 dilution was used as the secondary antibody.
    detail
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Product Information
Application
  • Applications Legend:
  • WB=Western Blot
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin-embedded Sections)
  • IHC-F=Immunohistochemistry (Frozen Sections)
  • IF=Immunofluorescence
  • FC=Flow Cytopmetry
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • E=ELISA
  • IP=Immunoprecipitation
  • DB=Dot Blot
  • CHIP=Chromatin Immunoprecipitation
  • FA=Fluorescence Assay
  • IEM=Immuno electron microscopy
  • EIA=Enzyme Immunoassay
FC, IHC-P, WB
Primary Accession Q13286
Other Accession Q60HH0
Reactivity Human, Mouse
Predicted Monkey
Host Rabbit
Clonality Polyclonal
Calculated MW H=48,43,46,35,38;M=48 KDa
Isotype Rabbit IgG
Antigen Source HUMAN
Additional Information
Gene ID 1201
Antigen Region 250-284 aa
Other Names Battenin, Batten disease protein, Protein CLN3, CLN3, BTS
Dilution WB~~1:1000
IHC-P~~1:25
FC~~1:25
Target/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.
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.
StorageMaintain 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.
PrecautionsCLN3 Antibody (Center) is for research use only and not for use in diagnostic or therapeutic procedures.
Protein Information
Name CLN3 (HGNC:2074)
Synonyms BTS
Function Mediates microtubule-dependent, anterograde transport connecting the Golgi network, endosomes, autophagosomes, lysosomes and plasma membrane, and participates in several cellular processes such as regulation of lysosomal pH, lysosome protein degradation, receptor- mediated endocytosis, autophagy, transport of proteins and lipids from the TGN, apoptosis and synaptic transmission (PubMed:10924275, PubMed:15471887, PubMed:18317235, PubMed:18817525, PubMed:20850431, PubMed:22261744). Facilitates the proteins transport from trans-Golgi network (TGN)-to other membrane compartments such as transport of microdomain-associated proteins to the plasma membrane, IGF2R transport to the lysosome where it regulates the CTSD release leading to regulation of CTSD maturation and thereby APP intracellular processing (PubMed:10924275, PubMed:18817525). Moreover regulates CTSD activity in response to osmotic stress (PubMed:23840424, PubMed:28390177). Also binds galactosylceramide and transports it from the trans Golgi to the rafts, which may have immediate and downstream effects on cell survival by modulating ceramide synthesis (PubMed:18317235). At the plasma membrane, regulates actin-dependent events including filopodia formation, cell migration, and pinocytosis through ARF1-CDC42 pathway and also the cytoskeleton organization through interaction with MYH10 and fodrin leading to the regulation of the plasma membrane association of Na+, K+ ATPase complex (PubMed:20850431). Regulates synaptic transmission in the amygdala, hippocampus, and cerebellum through regulation of synaptic vesicles density and their proximity to active zones leading to modulation of short-term plasticity and age-dependent anxious behavior, learning and memory (By similarity). Regulates autophagic vacuoles (AVs) maturation by modulating the trafficking between endocytic and autophagolysosomal/lysosomal compartments, which involves vesicle fusion leading to regulation of degradation process (By similarity). Participates also in cellular homeostasis of compounds such as, water, ions, amino acids, proteins and lipids in several tissue namely in brain and kidney through regulation of their transport and synthesis (PubMed:17482562).
Cellular Location Lysosome membrane; Multi-pass membrane protein. Late endosome. Lysosome. Golgi apparatus. Golgi apparatus membrane. Golgi apparatus, Golgi stack. Golgi apparatus, trans-Golgi network. Cell membrane Recycling endosome. Membrane raft. Membrane, caveola. Early endosome membrane. Synapse, synaptosome {ECO:0000250|UniProtKB:Q61124}. Late endosome membrane {ECO:0000250|UniProtKB:Q61124}. Cytoplasmic vesicle, autophagosome {ECO:0000250|UniProtKB:Q61124}. Note=CLN3 is not present in late endosomes/lysosomes in fibroblasts and neurons (PubMed:15240864) Trafficks from cell membrane to Golgi via endosomes (PubMed:15240864) Osmotic stress changes the subcellular localization of CLN3 (PubMed:23840424). Trafficks to intracellular compartments via the plasma membranet through AP3M1-dependent mechanisms (PubMed:14644441) Excluded from the synaptic 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 in the peripheral nerve (at protein level). Highly expressed in gray matter (at protein level)
Research Areas
Citations (0)
citation

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Background

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

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).

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$ 370.00
Cat# AW5327-U100
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