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CLN6 Antibody (PA5-106641) in ICC/IF
Immunocytochemistry analysis of CLN6 in HepG2 cells. The samples were fixed with PFA and permeabilized in 0.1% Triton X-100, then blocked in 10% serum for 45 minutes at 25°C. Samples were incubated with CLN6 polyclonal antibody (Product # PA5-106641) and mouse anti-beta tubulin antibody for 1 hour at 37°C. An AlexaFluor594 conjugated goat anti-rabbit IgG (Red) and an AlexaFluor488 conjugated goat anti-mouse IgG (Green) were used as the secondary antibodies. The nuclear counter stain is DAPI (blue).
产品信息
PA5-106641
应用
建议稀释比
已发表文章
产品规格
种属反应
Human,
Mouse,
Rat
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
A synthesized peptide derived from human CLN6(Accession Q9NWW5), corresponding to amino acid residues V225-L275.
偶联物
形式
Liquid
浓度
1 mg/mL
纯化类型
Affinity chromatography
保存液
PBS, pH 7.4, with 50% glycerol
内含物
0.02% sodium azide
保存条件
-20°C
运输条件
Wet ice
RRID
AB_2854309
产品详细信息
Antibody detects endogenous levels of total CLN6.
靶标信息
CLN6, a 311-amino acid protein, has 7 predicted transmembrane domains and is conserved across vertebrates. The CLN6 protein localizes to the endoplasmic reticulum but contributes to lysosomal function. Mutations in the CLN6 gene cause variant late-onset infantile neuronal ceroid lipofuscinosis (vLINCL), a lysosomal storage disorder marked by progressive mental deterioration and blindness; part of a group of severe inherited neurodegenerative disorders affecting children wherein lysosomes accumulate storage material, causing the death of neurons. CLN6 is one of eight proteins, including CLN1-8, that are associated with NCL.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
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生物信息学
蛋白别名: ceroid-lipofuscinosis; ceroid-lipofuscinosis neuronal 6 late infantile; Ceroid-lipofuscinosis neuronal protein 6; neuronal ceroid lipofuscinosis; Protein CLN6
基因别名: 1810065L06Rik; AW743417; CLN4A; CLN6; D9Bwg1455e; HsT18960; nclf
UniProt ID: (Human) Q9NWW5
Entrez Gene ID: (Human) 54982, (Mouse) 76524, (Rat) 315746
ganglioside metabolic process
lysosome organization
lysosomal lumen acidification
visual perception
cholesterol metabolic process
glycosaminoglycan metabolic process
locomotion involved in locomotory behavior
cellular macromolecule catabolic process
positive regulation of proteolysis
protein catabolic process
biological_process
