Anti-CLN6 antibody
| 英文名称 | CLN6 |
| 中文名称 | 神经细胞蜡样质脂褐质沉积病蛋白CLN6抗体 |
| 别 名 | Ceroid lipofuscinosis, neuronal 6, late infantile, variant; CLN6 protein; FLJ20561; Nclf; Protein CLN6; CLN6_HUMAN. |
DATASHEET
Host:Rabbit
Target Protein:CLN6
IR:Immunogen Range:101-200/311
Clonality:Polyclonal
Isotype:IgG
Entrez Gene:54982
Swiss Prot:Q9NWW5
Source:KLH conjugated synthetic peptide derived from human CLN6:101-200/311
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:?CLN6, a 311 amino acid protein, has seven 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.
Size:100ul
Concentration:1mg/ml
Applications:WB(1:500-2000)
ELISA(1:5000-10000)
IHC-P(1:100-500)
IHC-F(1:100-500)
IF(1:100-500)
Cross Reactive Species:Human
Mouse
Rat
Pig
Cow
Horse
Rabbit
Sheep
.
For research use only. Not intended for diagnostic or therapeutic use.

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