CLN3 Rabbit anti-Human, Polyclonal, Bioss

Catalog No.	Quantity
50-198-8202	100 μL

Catalog No. 50-198-8202 Supplier Bioss Supplier No. BS8016R

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: Immunofluorescence, Immunohistochemistry (Paraffin), Western Blot
• Classification: Polyclonal
• Concentration: 1 μg/mL
• Conjugate: Unconjugated
• Formulation: PBS with 1% BSA, 50% glycerol and 0.09% sodium azide; pH
• Gene: CLN3
• Gene Accession No.: Q13286
• Gene Alias: AI323623; Batten disease protein; Battenin; BTS; ceroid lipofuscinosis, neuronal 3, juvenile (Batten, Spielmeyer-Vogt disease); ceroid-lipofuscinosis, neuronal 3; CLN 3; Cln3; CLN3, battenin; Cln3p; JNCL; MGC102840; Protein CLN3
• Gene Symbols: CLN3
• Host Species: Rabbit
• Immunogen: KLH conjugated synthetic peptide derived from human CLN3.
• Purification Method: Protein A
• Quantity: 100 μL
• Regulatory Status: RUO
• Primary or Secondary: Primary
• Gene ID (Entrez): 1201
• Target Species: Human
• Content And Storage: -20°C
• Product Type: Antibody
• Form: Liquid
• Isotype: IgG