Anti-CLN5 antibody
英文名称 | CLN5 |
中文名称 | 神经细胞蜡样质脂褐质沉积病蛋白CLN5抗体 |
别 名 | Ceroid lipofuscinosis neuronal 5; Ceroid-lipofuscinosis neuronal protein 5; CLN5; CLN5_HUMAN; NCL; Protein CLN5. |
DATASHEET
Host:Rabbit
Target Protein:CLN5
IR:Immunogen Range:61-120/358
Clonality:Polyclonal
Isotype:IgG
Entrez Gene:1203
Swiss Prot:O75503
Source:KLH conjugated synthetic peptide derived from human CLN5:61-120/358
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:Neuronal ceroid-lipofuscinose (NCL), also designated Batten disease, comprises a group of recessively inherited, progressive neurodegenerative diseases found in children. NCL is characterized by atrophy of the brain and an accumulation of lysosome derived fluorescent bodies found in many cells, especially neurons. Symptoms of NCL include a failure of psychomotor development, seizures, impaired vision and premature death. The eight genes/proteins associated with NCL are designated CLN1-CLN8. Mutations in six of these genes results in a distinct type of NCL-disease; the six genes/proteins are CLN1 (encoding PPT1, a protein thiolesterase), CLN2 (encodeing the serine protease TPP1), CLN3, CLN5, CLN6 and CLN8. A single base duplication mutation in dog and cow CLN5 has been shown to cause NCL.
Size:100ul
Concentration:1mg/ml
Applications:WB(1:500-2000)
ELISA(1:5000-10000)
Cross Reactive Species:Human
Mouse
Rat
Pig
Cow
Horse
Rabbit
Sheep
.
For research use only. Not intended for diagnostic or therapeutic use.
VALIDATION IMAGES
Sample:
Hela(Human) Cell Lysate at 30 ug
HL60(Human) Cell Lysate at 30 ug
Primary: Anti- CLN5 (bs-11714R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 37 kD
Observed band size: 37 kD
Sample:
293T(Human) Cell Lysate at 30 ug
Primary: Anti- CLN5 (bs-11714R) at 1/1000 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 37 kD
Observed band size: 37 kD
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