Invitrogen

CLN3 Polyclonal Antibody

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CLN3 Antibody in Western Blot (WB)

CLN3 Antibody in Western Blot (WB)

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CLN3 Antibody (PA5-49382) in WB

Western blot analysis of CLN3 in various lysates. Samples were incubated with CLN3 polyclonal antibody (Product # PA5-49382) using a dilution of 1:500 followed by Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at a dilution of 1:10,000. Lysates/proteins: 20 µg per lane. Lane 1: Mouse kidney lysate; Lane 2: Neuro-2a whole cell lysate. Predicted band size: 48 kDa. Blocking/Dilution buffer: 5% NFDM/TBST.

CLN3 Antibody in Western Blot (WB)

CLN3 Antibody in Western Blot (WB)

CLN3 Antibody in Western Blot (WB)

CLN3 Antibody in Western Blot (WB)

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CLN3 Antibody in Western Blot (WB) — Western blot analysis of CLN3 in various lysates. Samples were incubated with CLN3 polyclonal antibody (Product # PA5-49382) using a dilution of 1:500 followed by Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at a dilution of 1:10,000. Lysates/proteins: 20 µg per lane. Lane 1: Mouse kidney lysate; Lane 2: Neuro-2a whole cell lysate. Predicted band size: 48 kDa. Blocking/Dilution buffer: 5% NFDM/TBST.

CLN3 Antibody in Western Blot (WB) — Western blot analysis of CLN3 in Neuro-2a whole cell lysate. Samples were incubated with CLN3 polyclonal antibody (Product # PA5-49382) using a dilution of 1:1,000 followed by Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at a dilution of 1:10,000. Lysates/proteins: 20 µg per lane. Predicted band size: 48 kDa. Blocking/Dilution buffer: 5% NFDM/TBST.

CLN3 Antibody in Western Blot (WB) — 2 FIGURE Protein samples from undifferentiated iPSC, 4-week CM, and 5-week NRO cultures were analysed by western blotting using an anti-CLN3 antibody and an anti-GAPDH antibody. LEIi004-A patient (P) iPSC and CM expressed lower levels of CLN3 protein than gene-corrected LEIi004-A-1 patient (GC) iPSC and CM. LEIi004-A-1 iPSC expressed similar levels of CLN3 protein as a control iPSC line (Con). Similar levels of CLN3 protein expression were detected in NRO from LEIi004-A and LEIi004-A-1

CLN3 Antibody in Western Blot (WB) — FIGURE 2 Protein samples from undifferentiated iPSC, 4-week CM, and 5-week NRO cultures were analysed by western blotting using an anti-CLN3 antibody and an anti-GAPDH antibody. LEIi004-A patient (P) iPSC and CM expressed lower levels of CLN3 protein than gene-corrected LEIi004-A-1 patient (GC) iPSC and CM. LEIi004-A-1 iPSC expressed similar levels of CLN3 protein as a control iPSC line (Con). Similar levels of CLN3 protein expression were detected in NRO from LEIi004-A and LEIi004-A-1

产品信息

PA5-49382

应用

建议稀释比

已发表文章

免疫印迹 (WB)

1:1000

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产品规格

种属反应

Human, Mouse

已发表种属

Human

宿主/亚型

Rabbit / IgG

分类

Polyclonal

类型

Antibody

抗原

KLH conjugated synthetic peptide between 250-284 amino acids from the Central region of human CLN3.

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偶联物

Unconjugated Unconjugated Unconjugated

形式

Liquid

浓度

0.4 mg/mL

纯化类型

Protein A, Antigen affinity chromatography

保存液

PBS, pH 7.4

内含物

0.09% sodium azide

保存条件

Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.

运输条件

Ambient (domestic); Wet ice (international)

RRID

AB_2634836

产品详细信息

Predicted to react with monkey based on sequence homology.

靶标信息

CLN3 is a highly glycosylated, hydrophobic, 438-amino acid protein with 6 transmembrane domains. The CLN3 protein localizes to the lysosomal membrane and plays a role in lysosomal function. It may act as a chaperone involved in the folding and unfolding of other proteins, namely subunit C of the ATP synthase complex. Mutations in the CLN3 gene cause Batten disease, a recessively inherited neurodegenerative disorder of childhood caused by lysosomal accumulation of hydrophobic material, mainly ATP synthase subunit C. Batten disease is the most common form of a group of disorders known as neuronal ceroid lipofuscinoses (NCLs). Symptoms of Batten disease include progressive loss of vision, seizures, and psychomotor disturbances.

仅用于科研。不用于诊断过程。未经明确授权不得转售。

Product References

Gene correction of the CLN3 c.175G>A variant in patient-derived induced pluripotent stem cells prevents pathological changes in retinal organoids.

Molecular genetics & genomic medicine

Zhang X,Zhang D,Thompson JA,Chen SC,Huang Z,Jennings L,McLaren TL,Lamey TM,De Roach JN,Chen FK,McLenachan S

Published figure using CLN3 polyclonal antibody (Product # PA5-49382) in Western Blot

Mon Mar 01 00:00:00 EST 2021

Gene correction of the CLN3 c.175G>A variant in patient-derived induced pluripotent stem cells prevents pathological changes in retinal organoids.

Molecular genetics & genomic medicine

Zhang X,Zhang D,Thompson JA,Chen SC,Huang Z,Jennings L,McLaren TL,Lamey TM,De Roach JN,Chen FK,McLenachan S

Published figure using CLN3 polyclonal antibody (Product # PA5-49382) in Western Blot

Mon Mar 01 00:00:00 EST 2021

生物信息学

蛋白别名: Batten disease protein; Battenin; ceroid-lipofuscinosis, neuronal 3; CLN 3; MGC102840; Protein CLN3

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基因别名: AI323623; BTS; CLN3; JNCL

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UniProt ID: (Human) Q9UP14, (Mouse) Q61124

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Entrez Gene ID: (Human) 1201, (Mouse) 12752

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protein binding unfolded protein binding calcium-dependent protein binding membrane trafficking regulatory protein membrane traffic protein

参与通路

action potential globoside metabolic process cellular amino acid metabolic process ceramide metabolic process glucosylceramide metabolic process galactosylceramide metabolic process sphingomyelin metabolic process receptor-mediated endocytosis vacuolar transport lysosome organization lysosomal lumen acidification associative learning arginine transport macroautophagy negative regulation of macroautophagy protein processing protein catabolic process ionotropic glutamate receptor signaling pathway lysosomal lumen pH elevation neurotransmitter metabolic process amyloid precursor protein catabolic process negative regulation of apoptotic process negative regulation of catalytic activity negative regulation of neuron apoptotic process negative regulation of proteolysis vesicle transport along microtubule neuromuscular process controlling balance regulation of cytosolic calcium ion concentration membrane organization autophagosome maturation positive regulation of protein phosphorylation transport amino acid transport endocytosis regulation of gene expression cell migration actin cytoskeleton organization regulation of filopodium assembly protein localization to membrane protein localization to plasma membrane