产品名称
CLN6 Rabbit Polyclonal Antibody
别名
CLN6; Ceroid-lipofuscinosis neuronal protein 6; Protein CLN6
蛋白名称
Ceroid-lipofuscinosis neuronal protein 6
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=54982
Human Swissprot No.
Q9NWW5
Human Swissprot Link
http://www.uniprot.org/uniprotkb/Q9NWW5/entry
免疫原
The antiserum was produced against synthesized peptide derived from human CLN6. AA range:221-270
特异性
CLN6 Polyclonal Antibody detects endogenous levels of CLN6 protein.
稀释度
WB 1:500 - 1:2000. IHC 1:100 - 1:300. ELISA: 1:40000.. IF 1:50-200
宿主
Polyclonal, Rabbit,IgG
背景介绍
This gene is one of eight which have been associated with neuronal ceroid lipofuscinoses (NCL). Also referred to as Batten disease, NCL comprises a class of autosomal recessive, neurodegenerative disorders affecting children. The genes responsible likely encode proteins involved in the degradation of post-translationally modified proteins in lysosomes. The primary defect in NCL disorders is thought to be associated with lysosomal storage function. [provided by RefSeq, Oct 2008],
组织表达
Epithelium,Lung,Urinary bladder,
细胞定位
Endoplasmic reticulum membrane ; Multi-pass membrane protein . Endoplasmic reticulum .
功能
disease:Defects in CLN6 are the cause of variant late-onset infantile neuronal ceroid lipofuscinosis (vLINCL) [MIM:601780].,online information:Neural Ceroid Lipofuscinoses mutation db,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
