CLN6 Rabbit anti-Human, Polyclonal, Bioss

Rabbit Polyclonal Antibody

Supplier:  Bioss BS8017R

Catalog No. 50-198-8203

Description

CLN6, a 311-amino acid protein, has 7 predicted transmembrane domains and is conserved across vertebrates. The CLN6 protein localizes to the endoplasmic reticulum but contributes to lysosomal function. Mutations in the CLN6 gene cause variant late-onset infantile neuronal ceroid lipofuscinosis (vLINCL), a lysosomal storage disorder marked by progressive mental deterioration and blindness; part of a group of severe inherited neurodegenerative disorders affecting children wherein lysosomes accumulate storage material, causing the death of neurons. CLN6 is one of eight proteins, including CLN1-8, that are associated with NCL.

Specifications

• Antigen: CLN6
• Classification: Polyclonal
• Conjugate: Unconjugated
• Gene: CLN6
• Gene Alias: 1810065L06Rik; AW743417; ceroid-lipofuscinosis neuronal 6 late infantile; Ceroid-lipofuscinosis neuronal protein 6; ceroid-lipofuscinosis, neuronal 6; ceroid-lipofuscinosis, neuronal 6, late infantile, variant; CLN4A; CLN6; D9Bwg1455e; HsT18960; nclf; neuronal ceroid lipofuscinosis; Protein CLN6
• Host Species: Rabbit
• Purification Method: Protein A
• Regulatory Status: RUO
• Gene ID (Entrez): 54982
• Content And Storage: -20°C
• Form: Liquid

• Applications: Immunofluorescence, Immunohistochemistry (Paraffin), Western Blot
• Concentration: 1 μg/mL
• Formulation: PBS with 1% BSA, 50% glycerol and 0.09% sodium azide; pH
• Gene Accession No.: Q9NWW5
• Gene Symbols: CLN6
• Immunogen: KLH conjugated synthetic peptide derived from human CLN6.
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human
• Product Type: Antibody
• Isotype: IgG