Target：CLN5

Fields：Lysosome

Gene Name：CLN5

Protein Name：Ceroid-lipofuscinosis neuronal protein 5

Human Gene Id：1203

Human Swiss Prot No：O75503

Mouse Gene Id：211286

Mouse Swiss Prot No：Q3UMW8

Immunogen：The antiserum was produced against synthesized peptide derived from human CLN5. AA range:171-220

Specificity：CLN5 Polyclonal Antibody detects endogenous levels of CLN5 protein.

Formulation：Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

Source：Polyclonal, Rabbit,IgG

Dilution：WB 1:500 - 1:2000. ELISA: 1:10000. Not yet tested in other applications.

Purification：The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration：1 mg/ml

Storage Stability：-15°C to -25°C/1 year(Do not lower than -25°C)

Other Name：CLN5;Ceroid-lipofuscinosis neuronal protein 5;Protein CLN5

Observed Band(KD)：48kD

Background：ceroid-lipofuscinosis, neuronal 5(CLN5) Homo sapiens 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],

Function：disease:Defects in CLN5 are the cause of ceroid lipofuscinosis neuronal 5 (CLN5) [MIM:256731]; also known as Finnish variant late-infantile neuronal ceroid lipofuscinosis (vLINCL). It is a fatal childhood neurodegenerative disease characterized by progressive visual and mental decline, motor disturbance, epilepsy and behavioral changes. The first symptom is motor clumsiness, followed by progressive visual failure, mental and motor deterioration and later by myoclonia and seizures.,online information:Neural Ceroid Lipofuscinoses mutation db,PTM:Glycosylated.,similarity:Belongs to the CLN5 family.,tissue specificity:Ubiquitous.,

Subcellular Location：[Ceroid-lipofuscinosis neuronal protein 5, secreted form]: Lysosome .; [Ceroid-lipofuscinosis neuronal protein 5]: Membrane ; Single-pass type II membrane protein . An amphipathic anchor region facilitates its association with the membrane. .

Expression：Ubiquitous.