Anti-CLN8 antibody
英文名称 | CLN8 |
中文名称 | 神经细胞蜡样质脂褐质沉积病蛋白CLN8抗体 |
别 名 | Ceroid-lipofuscinosis, neuronal 8 (epilepsy, progressive with mental retardation); Cln8; CLN8_HUMAN; EPMR; Protein CLN8. |
DATASHEET
Host:Rabbit
Target Protein:CLN8
IR:Immunogen Range:201-286/286
Clonality:Polyclonal
Isotype:IgG
Entrez Gene:2055
Swiss Prot:Q9UBY8
Source:KLH conjugated synthetic peptide derived from human CLN8:201-286/286
Purification:affinity purified by Protein A
Storage:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Background:CLN8, a 286 amino acid transmembrane protein, localizes mainly to the endoplasmic reticulum, but also partially to the ER-Golgi intermediate compartment (ERGIC). Mutations in the CLN8 gene cause neuronal ceroid lipofuscinosis 8 and progressive epilepsy with mental retardation (EPMR). Both disorders are forms of neuronal ceroid-lipofuscinose (NCL), a group of progressive neurodegenerative diseases found in children, characterized by failure of psychomotor development, impaired vision, seizures and premature death. The CLN8 protein is one of eight proteins in the CLN family, including CLN1-CLN7, which are associated with NCL.
Size:100ul
Concentration:1mg/ml
Applications:WB(1:500-2000)
ELISA(1:5000-10000)
IHC-P(1:100-500)
IHC-F(1:100-500)
ICC(1:100-500)
IF(1:100-500)
Cross Reactive Species:Human
Mouse
Rat
Dog
Pig
Horse
Rabbit
.
For research use only. Not intended for diagnostic or therapeutic use.
VALIDATION IMAGES
Sample:
Hela(Human) Cell Lysate at 30 ug
Primary: Anti- CLN8 (bs-11715R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 33 kD
Observed band size: 33 kD
Tissue/cell: Rat brain tissue; 4% Paraformaldehyde-fixed and paraffin-embedded;
Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;
Incubation: Anti-CLN8 Polyclonal Antibody, Unconjugated(bs-11715R) 1:500, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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