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CLN3 encodes a protein that is involved in lysosomal function. Zusätzlich bieten wir Ihnen CLN3 Antikörper (38) und CLN3 Proteine (4) und viele weitere Produktgruppen zu diesem Protein an.
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
The lysosomal enzyme cathepsin D (CTSD (zeige CTSD ELISA Kits)) mediates the proteolytic cleavage of PSAP (zeige PSAP ELISA Kits) precursor into saposins A-D. Myc (zeige MYC ELISA Kits)-CLN3 colocalized with CTSD (zeige CTSD ELISA Kits) and activity of CTSD (zeige CTSD ELISA Kits) decreased as myc (zeige MYC ELISA Kits)-CLN3 expression increased, and clearly decreased under hyperosmotic conditions
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.
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.
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 (zeige CA2 ELISA Kits)+) handling and in autophagic pathway flux and establish a powerful new platform for therapeutic screening.
CLN3 mutation is associated with neuronal ceroid lipofuscinosis (zeige CLN6 ELISA Kits).
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 (zeige RACGAP1 ELISA Kits) Cdc42 (zeige CDC42 ELISA Kits) is misregulated in the absence of CLN3, and thus may be a common link to multiple cellular defects.
Rssults suggested that CLN2 (zeige TPP1 ELISA Kits), CLN3 and CLN5 (zeige CLN5 ELISA Kits) 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 (zeige GRIN1 ELISA Kits) receptor function in Cln3(Deltaex7/8) neurons and suggest that both AMPA (zeige GRIA3 ELISA Kits) and NMDA receptors are potential therapeutic targets in juvenile neuronal ceroid lipofuscinosis (zeige CLN6 ELISA Kits)
The migration defect in Cln3(-/-) results, in part, from the loss of the CLN3-myosin-IIB (zeige MYH10 ELISA Kits) 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 (zeige CLN6 ELISA Kits) (JNCL; Batten disease) mutation exhibit progressive neurologic disease that begins before birth.
The candidate genes for bovine Neuronal Ceroid Lipofuscinosis (zeige CLN6 ELISA Kits), CLN3, CLN5 (zeige CLN5 ELISA Kits) and CLN6 (zeige CLN6 ELISA Kits), 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.
batten disease protein
, 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