Anti-CLN3 antibody (ab87438)

Overview

• Product nameAnti-CLN3 antibodySee all CLN3 primary antibodies ...
• Description
  Rabbit polyclonal to CLN3
• Tested applicationsWB, ICC/IF more details
• Species reactivity
  Reacts with: Mouse
  Predicted to work with: Rat, Human, Pig
• Immunogen

  Synthetic peptide conjugated to KLH derived from within residues 50 - 150 of Human CLN3.

  Read Abcam's proprietary immunogen policy

  (Peptide available as ab87439.)

• Positive controlThis antibody gave a positive signal in Mouse Brain Tissue Lysate.

Properties

• FormLiquid
• Storage instructionsStore at +4°C short term (1-2 weeks). Aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.
• Storage bufferPreservative: 0.02% Sodium Azide
  Constituents: 1% BSA, PBS, pH 7.4
• Concentration information loading...
• PurityImmunogen affinity purified
• Clonality Polyclonal
• IsotypeIgG
• Research Areas
  • Neuroscience
  • Neurology process
  • Neurodegenerative disease
  • Other

  • Neuroscience
  • Neurotransmission
  • Secretory Vesicles
  • Other

  • Cell Biology
  • Other Antibodies
  • Other Antibodies

Applications

Target

• FunctionInvolved in microtubule-dependent, anterograde transport of late endosomes and lysosomes.
• Involvement in diseaseDefects in CLN3 are the cause of neuronal ceroid lipofuscinosis type 3 (CLN3) [MIM:204200]; 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.
• Sequence similaritiesBelongs to the battenin family.
• Post-translational
  modificationsHighly glycosylated.
  Farnesylation is important for trafficking to lysosomes.
• Cellular localizationLysosome membrane. Late endosome.

Target information above from: UniProt accession Q13286 The UniProt Consortium
The Universal Protein Resource (UniProt) in 2010
Nucleic Acids Res. 38:D142-D148 (2010) .

Information by UniProt
• Database links
  • Entrez Gene: 1201 Human
  • Entrez Gene: 12752 Mouse
  • Entrez Gene: 293485 Rat
  • Omim: 607042 Human
  • SwissProt: Q13286 Human
  • SwissProt: Q61124 Mouse
  • Unigene: 534667 Human
  • Unigene: 268930 Mouse

• Alternative names
  Batten disease protein antibodyBattenin antibodyBTS antibody

  Ceroid lipofuscinosis neuronal 3 antibodyCeroid lipofuscinosis neuronal 3 juvenile (Batten Spielmeyer Vogt disease) antibodyCeroid lipofuscinosis neuronal 3 juvenile antibodyCLN 3 antibodyCLN3 antibodyCLN3_HUMAN antibodyJNCL antibodyMGC102840 antibodyProtein CLN3 antibody

  see all

Anti-CLN3 antibody images

• 

  Western blot - Anti-CLN3 antibody (ab87438)
  Anti-CLN3 antibody (ab87438) at 1 µg/ml + Brain (Mouse) Tissue Lysate at 10 µg
  
  Secondary
  Goat polyclonal to Rabbit IgG - H&L - Pre-Adsorbed (HRP) at 1/3000 dilution
  developed using the ECL technique
  
  Performed under reducing conditions.
  
  Predicted band size : 48 kDa
  Observed band size : 52 kDa (why is the actual band size different from the predicted?)
  
• 

  Immunocytochemistry/ Immunofluorescence - CLN3 antibody (ab87438)
  ICC/IF image of ab87438 stained SHSY5Y cells. The cells were 4% formaldehyde fixed (10 min) and then incubated in 1%BSA / 10% normal goat serum / 0.3M glycine in 0.1% PBS-Tween for 1h to permeabilise the cells and block non-specific protein-protein interactions. The cells were then incubated with the antibody (ab87438, 5µg/ml) overnight at +4°C. The secondary antibody (green) was Alexa Fluor® 488 goat anti-rabbit IgG (H+L) used at a 1/1000 dilution for 1h. Alexa Fluor® 594 WGA was used to label plasma membranes (red) at a 1/200 dilution for 1h. DAPI was used to stain the cell nuclei (blue) at a concentration of 1.43µM.