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CLN3 encodes a protein that is involved in lysosomal function. Additionally we are shipping CLN3 Proteins (4) and many more products for this protein.
Showing 10 out of 37 products:
Human Monoclonal CLN3 Primary Antibody for WB - ABIN393265
Adams, Beck, Levy, Jordan, Kwon, Marshall, Vierhile, Augustine, de Blieck, Pearce, Mink: Genotype does not predict severity of behavioural phenotype in juvenile neuronal ceroid lipofuscinosis (Batten disease). in Developmental medicine and child neurology 2010
Show all 5 references for ABIN393265
Our subcellular localization study in neurons refines the localization and subcellular targeting of CLN3 to late endosomal-lysosomal compartments
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
The small GTPase (show RACGAP1 Antibodies) Cdc42 (show CDC42 Antibodies) is misregulated in the absence of CLN3, and thus may be a common link to multiple cellular defects.
Rssults suggested that CLN2 (show TPP1 Antibodies), CLN3 and CLN5 (show CLN5 Antibodies) genes may play an important role in early embryonal neurogenesis.
The aim of our study was to investigate the visual disease progression in the Cln3 (Deltaex7/8) mice.
CLN3 facilitates transport of microdomain-associated proteins.
results demonstrate altered glutamate (show GRIN1 Antibodies) receptor function in Cln3(Deltaex7/8) neurons and suggest that both AMPA (show GRIA3 Antibodies) and NMDA receptors are potential therapeutic targets in juvenile neuronal ceroid lipofuscinosis (show CLN6 Antibodies)
The migration defect in Cln3(-/-) results, in part, from the loss of the CLN3-myosin-IIB (show MYH10 Antibodies) interaction.
Findings suggest an osmoregulated role for CLN3p in renal medullary control of water and K(+) balance.
Cln3(Deltaex7/8) knock-in mice with the common juvenile-onset neuronal ceroid lipofuscinosis (show CLN6 Antibodies) (JNCL; Batten disease) mutation exhibit progressive neurologic disease that begins before birth.
CLN3 knockdown inhibits cell proliferation and induces G0/G1 cell cycle arrest in the A2780 cell line and its drug-resistant sub-lines.
The membrane topology of human CLN3 protein.
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.
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
These results further support an important role for the CLN3 protein in intracellular Ca(2 (show CA2 Antibodies)+) handling and in autophagic pathway flux and establish a powerful new platform for therapeutic screening.
CLN3 mutation is associated with neuronal ceroid lipofuscinosis (show CLN6 Antibodies).
Genetic testing for CLN3 should be considered in autophagic vacuolar myopathy (AVM), with autophagic vacuoles and sarcolemmal features.
CLN3 was identified as a novel disease gene for non-syndromic retinal diseases as supported by five unrelated patient families in this study.
CLN3 is involved in the response and adaptation to cellular stress.
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.
The candidate genes for bovine Neuronal Ceroid Lipofuscinosis (show CLN6 Antibodies), CLN3, CLN5 (show CLN5 Antibodies) and CLN6 (show CLN6 Antibodies), have been mapped to facilitate linkage analysis in cattle and sheep.
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.
, ceroid-lipofuscinosis, neuronal 3
, ceroid-lipofuscinosis, neuronal 3, juvenile (Batten, Spielmeyer-Vogt disease)
, ceroid-lipofuscinosis, neuronal 3, juvenile
, batten disease protein
, ceroid lipofuscinosis, neuronal 3, juvenile (Batten, Spielmeyer-Vogt disease)