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Rabbit Anti-CLN6 Recombinant Antibody (EG735) (CBMAB-EN889-LY)

The product is antibody recognizes CLN6. The antibody EG735 immunoassay techniques such as: WB: 1:500~1:1000 IHC: 1:50~1:100 ELISA: 1:40000.
See all CLN6 antibodies

Summary

Host Animal
Rabbit
Specificity
Human
Clone
EG735
Antibody Isotype
IgG
Application
WB: 1:500~1:1000 IHC: 1:50~1:100 ELISA: 1:40000

Basic Information

Immunogen
The antibody was produced against synthesized peptide derived from internal of human CLN6.
Specificity
Human
Antibody Isotype
IgG
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
CLN6, Transmembrane ER Protein
Introduction
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]
Entrez Gene ID
54982
UniProt ID
Q9NWW5
Alternative Names
CLN6, Transmembrane ER Protein; Ceroid-Lipofuscinosis, Neuronal 6, Late Infantile, Variant; Ceroid-Lipofuscinosis, Neuronal 6, Late Infantile; Ceroid-Lipofuscinosis Neuronal 6 Late Infantile; Ceroid-Lipofuscinosis Neuronal Protein 6; Protein CLN6; HsT18960; CLN4A; Nclf;
Biological Process
Cellular macromolecule catabolic process Source: Ensembl
Cholesterol metabolic process Source: UniProtKB
Ganglioside metabolic process Source: UniProtKB
Glycosaminoglycan metabolic process Source: UniProtKB
Locomotion involved in locomotory behavior Source: Ensembl
Lysosomal lumen acidification Source: UniProtKB
Lysosome organization Source: GO_Central
Positive regulation of proteolysis Source: UniProtKB
Protein catabolic process Source: UniProtKB
Visual perception Source: Ensembl
Cellular Location
Endoplasmic reticulum membrane; Endoplasmic reticulum
Involvement in disease
Ceroid lipofuscinosis, neuronal, 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.
Ceroid lipofuscinosis, neuronal, 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.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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