anti-Ceroid-Lipofuscinosis, Neuronal 3 (CLN3) Antibodies

CLN3 encodes a protein that is involved in lysosomal function. Additionally we are shipping CLN3 Proteins (4) and and many more products for this protein.

list all antibodies Gene Name GeneID UniProt
CLN3 1201 Q13286
CLN3 12752 Q61124
CLN3 293485  
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Top anti-CLN3 Antibodies at antibodies-online.com

Showing 10 out of 41 products:

Catalog No. Reactivity Host Conjugate Application Images Quantity Supplier Delivery Price Details
Human Rabbit Un-conjugated IHC, WB 100 μL Log in to see 11 to 13 Days
$366.77
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Human Rabbit Un-conjugated ELISA, WB   100 μL Log in to see 11 to 13 Days
$335.04
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Human Rabbit FITC IF (p)   100 μL Log in to see 14 to 21 Days
$416.90
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Human Rabbit Alexa Fluor 350 IF (p)   100 μL Log in to see 14 to 21 Days
$416.90
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Human Rabbit Alexa Fluor 488 IF (p)   100 μL Log in to see 14 to 21 Days
$416.90
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Human Rabbit Alexa Fluor 555 IF (p)   100 μL Log in to see 14 to 21 Days
$416.90
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Human Rabbit Cy5 IF (p)   100 μL Log in to see 14 to 21 Days
$416.90
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Human Rabbit Cy7 IF (p)   100 μL Log in to see 14 to 21 Days
$416.90
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Human Rabbit Cy3 IF (p)   100 μL Log in to see 14 to 21 Days
$416.90
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Human Rabbit Alexa Fluor 647 IF (p)   100 μL Log in to see 14 to 21 Days
$416.90
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More Antibodies against CLN3 Interaction Partners

Human Ceroid-Lipofuscinosis, Neuronal 3 (CLN3) interaction partners

  1. Our case study shows that (1) non-syndromic CLN3 disease leads to rod and delayed primary cone degeneration resulting in constricting peripheral field and enlarging central scotoma and, (2) the c.175G>A CLN3 mutation, altered splicing of the CLN3 gene.

  2. the results of this study indicate that Cln3 functions in both conventional and unconventional protein secretion and that loss of Cln3 results in deregulated secretion during early development. Importantly, this is the first evidence in any system linking CLN3 function to protein secretion.

  3. AAV2-CLN3 was efficacious in restoring full-length CLN3 transcript and protein in patient-specific fibroblasts and iPSC-derived retinal neurons. When injected into the subretinal space of wild-type mice, purified AAV2-CLN3 did not show any evidence of retinal toxicity

  4. The lysosomal enzyme cathepsin D (CTSD) mediates the proteolytic cleavage of PSAP precursor into saposins A-D. Myc-CLN3 colocalized with CTSD and activity of CTSD decreased as myc-CLN3 expression increased, and clearly decreased under hyperosmotic conditions

  5. This is the first detailed morphological evaluation of CLN3 patients in the first years after the subjective onset of ocular symptoms. CLN3 is characterized by an early degeneration predominant of the first and second neuron compared to other macular and generalized retinal dystrophies.

  6. The age at onset and natural progression of retinal disease differs greatly between syndromic and nonsyndromic CLN3 disease, which may be associated with genotypic differences.

  7. CLN3 knockdown inhibits cell proliferation and induces G0/G1 cell cycle arrest in the A2780 cell line and its drug-resistant sub-lines.

  8. The membrane topology of human CLN3 protein.

  9. The eyes and vision of heterozygous carriers of CLN3 disease showed normal features when compared to a control group, which controverts a previously suggested retinal dysfunction in these subjects.

  10. This new model system, which allows for the study of Cln3 function in both single cells and a multicellular organism, together with the observation that expression of human CLN3 restores abnormalities in Dictyostelium cln3- cells

  11. These results further support an important role for the CLN3 protein in intracellular Ca(2+) handling and in autophagic pathway flux and establish a powerful new platform for therapeutic screening.

  12. CLN3 mutation is associated with neuronal ceroid lipofuscinosis.

  13. Genetic testing for CLN3 should be considered in autophagic vacuolar myopathy (AVM), with autophagic vacuoles and sarcolemmal features.

  14. CLN3 was identified as a novel disease gene for non-syndromic retinal diseases as supported by five unrelated patient families in this study.

  15. CLN3 is involved in the response and adaptation to cellular stress.

  16. Protein interaction mapping analysis suggests CLN3 is involved in transmembrane transport, lipid homeostasis, neuronal excitability and link it to G-protein signaling and protein folding/sorting in the endoplasmic reticulum.

  17. The data presented in this study provide novel insights into the role of CLN3 in late endosomal/lysosomal membrane transport.

  18. Btn1 controls retrograde sorting by regulating SNARE phosphorylation and assembly, a process that may be adversely affected in Batten Disease patients.

  19. The predominant distribution of CLN3 reporter neurons in visual, limbic and subcortical motor structures of transgenic mice correlates well with the clinical symptoms of juvenile neuronal ceroid lipofuscinosis.

  20. CLN6 and CLN3 mutations trigger distinct processes that converge on a shared pathway, which is responsible for proper subunit c protein turnover and neuronal cell survival.

Mouse (Murine) Ceroid-Lipofuscinosis, Neuronal 3 (CLN3) interaction partners

  1. These results show that CLN3 deficiency alters antigen presenting cells, which can be a major contributor to the autoimmune response in juvenile neuronal ceroid lipofuscinosis.

  2. Loss of CLN3 protein leads to early presynaptic changes and altered short-term plasticity in a mouse model of juvenile Batten disease.

  3. Our subcellular localization study in neurons refines the localization and subcellular targeting of CLN3 to late endosomal-lysosomal compartments

  4. findings in two distinct lysosome-related degradative pathways constitute the first description of a deficit in the retinal pigment epithelium caused by loss of CLN3

  5. The small GTPase Cdc42 is misregulated in the absence of CLN3, and thus may be a common link to multiple cellular defects.

  6. Rssults suggested that CLN2, CLN3 and CLN5 genes may play an important role in early embryonal neurogenesis.

  7. The aim of our study was to investigate the visual disease progression in the Cln3 (Deltaex7/8) mice.

  8. CLN3 facilitates transport of microdomain-associated proteins.

  9. results demonstrate altered glutamate receptor function in Cln3(Deltaex7/8) neurons and suggest that both AMPA and NMDA receptors are potential therapeutic targets in juvenile neuronal ceroid lipofuscinosis

  10. The migration defect in Cln3(-/-) results, in part, from the loss of the CLN3-myosin-IIB interaction.

  11. Findings suggest an osmoregulated role for CLN3p in renal medullary control of water and K(+) balance.

  12. Cln3(Deltaex7/8) knock-in mice with the common juvenile-onset neuronal ceroid lipofuscinosis (JNCL; Batten disease) mutation exhibit progressive neurologic disease that begins before birth.

  13. we investigated endogenous Cln3p expression using two peptide antibodies raised against two distinct epitopes of murine Cln3p.

  14. CLN3 defect may result in altered mitochondrial function in eye but not other tissue.

  15. The Cln3 null mutant (Cln3(-/-)) mouse model of juvenile NCL (JNCL) that revealed a delayed onset neurodegenerative phenotype with no significant regional atrophy, but with widespread loss of hippocampal interneurons that was at 14 months of age.

  16. AP-1 and AP-3 facilitate lysosomal targeting of Batten disease protein CLN3 via its dileucine motif

  17. Cnl3 mutant mice revealed a pronounced loss of neurons within individual laminae of somatosensory cortex of affected mice and the novel finding of a loss of sensory relay thalamic neurons.

  18. cln3 has a role in pathology of Batten disease, as shown by NMR metabolomic analysis of the mouse model

  19. Having previously demonstrated that Cln3(-/-) mice have decreased optic nerve axonal density, we now demonstrate a decrease in nerve conduction.

  20. Vulnerability of CLN3 knock-out or CLN3 deletion (1-153)-expressing neuronal cells to Ca(2+)-induced cell death may be mediated by calsenilin.

Cow (Bovine) Ceroid-Lipofuscinosis, Neuronal 3 (CLN3) interaction partners

  1. The candidate genes for bovine Neuronal Ceroid Lipofuscinosis, CLN3, CLN5 and CLN6, have been mapped to facilitate linkage analysis in cattle and sheep.

CLN3 Antigen Profile

Protein Summary

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.

Gene names and symbols associated with CLN3

  • CLN3, battenin (CLN3) antibody
  • ceroid lipofuscinosis, neuronal 3, juvenile (Batten, Spielmeyer-Vogt disease) (Cln3) antibody
  • ceroid-lipofuscinosis, neuronal 3 (cln3) antibody
  • battenin (PTRG_05620) antibody
  • CLN3, battenin (Cln3) antibody
  • battenin (LOC410860) antibody
  • ceroid-lipofuscinosis, neuronal 3 L homeolog (cln3.L) antibody
  • uncharacterized LOC467935 (LOC467935) antibody
  • AI323623 antibody
  • bts antibody
  • GB12798 antibody
  • JNCL antibody
  • MGC80041 antibody
  • zgc:92244 antibody

Protein level used designations for CLN3

batten disease protein , battenin , Cln3p , ceroid lipofuscinosis, neuronal 3, juvenile (Batten, Spielmeyer-Vogt disease) , ceroid-lipofuscinosis, neuronal 3 , ceroid-lipofuscinosis, neuronal 3, juvenile (Batten, Spielmeyer-Vogt disease) , ceroid-lipofuscinosis, neuronal 3, juvenile , battenin-like

GENE ID SPECIES
1201 Homo sapiens
12752 Mus musculus
492340 Danio rerio
6343873 Pyrenophora tritici-repentis Pt-1C-BFP
293485 Rattus norvegicus
410860 Apis mellifera
446498 Xenopus laevis
467935 Pan troglodytes
479794 Canis lupus familiaris
504799 Bos taurus
100009244 Oryctolagus cuniculus
100124328 Xenopus (Silurana) tropicalis
100127354 Sus scrofa
100388249 Callithrix jacchus
100457403 Pongo abelii
100583151 Nomascus leucogenys
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