CLN3 rabbit pAb

This gene encodes a protein that is involved in lysosomal function. Mutations in this, as well as other neuronal ceroid-lipofuscinosis (CLN) genes, cause neurodegenerative diseases commonly known as Batten disease or collectively known as neuronal ceroid lipofuscinoses (NCLs). Many alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Jul 2008],

Product Specifications

Background

This gene encodes a protein that is involved in lysosomal function. Mutations in this, as well as other neuronal ceroid-lipofuscinosis (CLN) genes, cause neurodegenerative diseases commonly known as Batten disease or collectively known as neuronal ceroid lipofuscinoses (NCLs) . Many alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Jul 2008]

UniProt

Q13286

Swiss Prot

Q13286

Reactivity

Human; Rat; Mouse

Immunogen

Synthesized peptide derived from human protein . at AA range: 221-270

Clonality

Polyclonal

Source

Rabbit

Applications

WB; ELISA

Concentration

1 mg/ml

Dilution

WB 1:500-2000 ELISA 1:5000-20000

Molecular Weight

48kD

Storage Conditions

-20°C/1 year

Observed Molecular Weight

48kD

Fragment

IgG

Subcellular Location

Lysosome membrane ; Multi-pass membrane protein . Late endosome . Lysosome . Golgi apparatus . Golgi apparatus membrane . Golgi apparatus, Golgi stack . Golgi apparatus, trans-Golgi network . Cell membrane . Recycling endosome . Membrane raft . Membrane, caveola . Early endosome membrane . Cell junction, synapse, synaptosome . Late endosome membrane . Cytoplasmic vesicle, autophagosome . CLN3 is not present in late endosomes/lysosomes in fibroblasts and neurons (PubMed:15240864) . Trafficks from cell membrane to Golgi via endosomes (PubMed:15240864) . Osmotic stress changes the subcellular localization of CLN3 (PubMed:23840424) . Trafficks to intracellular compartments via the plasma membranet through AP3M1-dependent mechanisms (PubMed:14644441) . Excluded from the synaptic vesicles (By simila