CLN3 Rabbit anti-Human, Mouse, Rat, Polyclonal, Proteintech

Catalog No.	Quantity
50-173-2861	150 μL

Catalog No. 50-173-2861 Supplier Proteintech Group Inc Supplier No. 203861AP150UL

Description

Rabbit Polyclonal Antibody

CLN3 is a highly glycosylated, hydrophobic, 438-amino acid protein with 6 transmembrane domains. The CLN3 protein localizes to the lysosomal membrane and plays a role in lysosomal function. It may act as a chaperone involved in the folding and unfolding of other proteins, namely subunit C of the ATP synthase complex. Mutations in the CLN3 gene cause Batten disease, a recessively inherited neurodegenerative disorder of childhood caused by lysosomal accumulation of hydrophobic material, mainly ATP synthase subunit C. Batten disease is the most common form of a group of disorders known as neuronal ceroid lipofuscinoses (NCLs). Symptoms of Batten disease include progressive loss of vision, seizures, and psychomotor disturbances.

Specifications

• Antigen: CLN3
• Applications: Immunocytochemistry, Immunofluorescence, Immunohistochemistry (Paraffin), Western Blot
• Classification: Polyclonal
• Concentration: 0.25 mg/mL
• Conjugate: Unconjugated
• Formulation: PBS with 50% glycerol and 0.02% sodium azide; pH 7.3
• Gene: CLN3
• Gene Accession No.: Q13286, Q61124
• Gene Alias: Batten disease protein, BATTENIN, BTS, CLN3, JNCL, Protein CLN3
• Gene Symbols: CLN3
• Host Species: Rabbit
• Immunogen: CLN3 Fusion Protein Ag14224
• Purification Method: Antigen Affinity Chromatography
• Quantity: 150 μL
• Regulatory Status: RUO
• Primary or Secondary: Primary
• Gene ID (Entrez): 1201, 12752, 293485
• Target Species: Human, Mouse, Rat
• Content And Storage: -20°C
• Product Type: Antibody
• Form: Liquid
• Isotype: IgG