Rabbit anti-Human CLN6 Polyclonal Antibody | anti-CLN6 antibody

CLN6 Antibody

Cat. Num. AAA9412546

• Gene Names: CLN6; nclf; CLN4A; HsT18960
• Reactivity: Human
• Applications: Western Blot, Immunohistochemistry
• Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
• Synonyms: CLN6; Polyclonal Antibody; CLN6 Antibody; ceroid-lipofuscinosis neuronal protein 6; anti-CLN6 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Specificity: The antibody detects endogenous levels of total CLN6 protein.
• Purity/Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
• Form/Format: Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
• Concentration: 1.0 mg/ml (varies by lot)
• Sequence Length: 311

• Applicable Applications for anti-CLN6 antibody: Western Blot (WB), Immunohistochemistry (IHC)
• Application Notes: Western blotting: 1:500~1:3000; Immunohistochemistry: 1:50~1:100
• Immunogen Type: Peptide
• Immunogen Description: Synthesized peptide derived from internal of human CLN6.
• Target Name: CLN6
• Preparation and Storage: Store at -20 degree C

Western Blot (WB)

(Western blot analysis of extracts from HeLa cells, using CLN6 antibody.)

Immunohistochemistry (IHC)

(Immunohistochemistry analysis of paraffin-embedded human cervix tissue using CLN6 antibody.)

Related Product Information for anti-CLN6 antibody

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.Ota T., Nat. Genet. 36:40-45(2004).The MGC Project Team; Genome Res. 14:2121-2127(2004).Mole S.E., Exp. Cell Res. 298:399-406(2004).

Product Categories/Family for anti-CLN6 antibody

Total protein Ab

NCBI and Uniprot Product Information

• NCBI GI #: 8923532
• NCBI GeneID: 54982
• NCBI Accession #: NP_060352.1
• NCBI GenBank Nucleotide #: NM_017882.2
• UniProt Accession #: Q9NWW5; Q6IAB1; Q96SR0; A8K560; B4DDH6
• Molecular Weight: 39,484 Da
• NCBI Official Full Name: ceroid-lipofuscinosis neuronal protein 6
• NCBI Official Synonym Full Names: ceroid-lipofuscinosis, neuronal 6, late infantile, variant
• NCBI Official Symbol: CLN6
• NCBI Official Synonym Symbols: nclf; CLN4A; HsT18960
• NCBI Protein Information: ceroid-lipofuscinosis neuronal protein 6
• UniProt Protein Name: Ceroid-lipofuscinosis neuronal protein 6
• Protein Family: Ceroid-lipofuscinosis neuronal protein
• UniProt Gene Name: CLN6
• UniProt Synonym Gene Names: Protein CLN6
• UniProt Entry Name: CLN6_HUMAN

NCBI Description

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]

Uniprot Description

CLN6: Defects in CLN6 are the cause of neuronal ceroid lipofuscinosis type 6 (CLN6). A form of neuronal ceroid lipofuscinosis. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by intracellular accumulation of autofluorescent liposomal material, and clinically by seizures, dementia, visual loss, and/or cerebral atrophy. The lipopigment patterns observed most often in neuronal ceroid lipofuscinosis type 6 comprise mixed combinations of granular, curvilinear, and fingerprint profiles. Defects in CLN6 are the cause of neuronal ceroid lipofuscinosis type 4A (CLN4A). An adult-onset neuronal ceroid lipofuscinosis. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by intracellular accumulation of autofluorescent liposomal material, and clinically by seizures, dementia, visual loss, and/or cerebral atrophy. CLN4A has no visual involvement and is characterized by progressive myoclonic epilepsy.

Protein type: Membrane protein, multi-pass; Membrane protein, integral

Chromosomal Location of Human Ortholog: 15q23

Cellular Component: nucleoplasm; endoplasmic reticulum membrane; nuclear membrane; intracellular membrane-bound organelle; membrane; endoplasmic reticulum lumen; endoplasmic reticulum; integral to membrane

Molecular Function: protein binding; protein homodimerization activity

Biological Process: cholesterol metabolic process; glycosaminoglycan metabolic process; visual perception; positive regulation of proteolysis; lysosomal lumen acidification; protein catabolic process; cellular macromolecule catabolic process; locomotion during locomotory behavior; ganglioside metabolic process

Disease: Ceroid Lipofuscinosis, Neuronal, 6; Ceroid Lipofuscinosis, Neuronal, 4a, Autosomal Recessive