Anti-CLN3 antibody (ab87438)
- Product nameAnti-CLN3 antibodySee all CLN3 primary antibodies ...
- DescriptionRabbit polyclonal to CLN3
- Tested applicationsWB, ICC/IF more details
- Species reactivityReacts with: Mouse
Predicted to work with: Rat, Human, Pig
Synthetic peptide conjugated to KLH derived from within residues 50 - 150 of Human CLN3.
(Peptide available as ab87439.)
- Positive controlThis antibody gave a positive signal in Mouse Brain Tissue Lysate.
- 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
- Research Areas
Our Abpromise guarantee covers the use of ab87438 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||WB: Use a concentration of 1 µg/ml. Detects a band of approximately 52 kDa (predicted molecular weight: 48 kDa).Can be blocked with CLN3 peptide (ab87439).|
|ICC/IF||ICC/IF: Use a concentration of 5 µg/ml.|
- 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.
Farnesylation is important for trafficking to lysosomes.
- Cellular localizationLysosome membrane. Late endosome.
- 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
Anti-CLN3 antibody images
Anti-CLN3 antibody (ab87438) at 1 µg/ml + Brain (Mouse) Tissue Lysate at 10 µg
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?)
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.
References for Anti-CLN3 antibody (ab87438)
ab87438 has not yet been referenced specifically in any publications.