Product Description

CLN5 Rabbit Polyclonal Antibody

Sizes: 50μL, 100μL

Catalogue Numbers: ES4682-50, ES4682-100

Lead times: 1-2 weeks, if manufacturer has product in stock

Manufacturer/Ship Location: China

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],

Reactivity: Human, Mouse, Rat

Host: Rabbit

Applications: WB, ELISA

Alternate Names: CLN5, Ceroid-lipofuscinosis neuronal protein 5, Protein CLN5

Clonality: Polyclonal

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

Isotype: IgG

Target: CLN5

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

Dilution: Western Blot: 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,

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

Expression: Ubiquitous,

Observed Band (KD): 48kD

Fields: Lysosome

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

Storage and Stability: -20°C/1 year

Gene Name: CLN5

Protein Name: Ceroid-lipofuscinosis neuronal protein 5

Mouse Gene ID: 211286

Mouse SwissProt Number: Q3UMW8

Research Use Only