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.
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.
