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

Name: Mouse Anti-CLN6 Recombinant Antibody (EG735)
SKU: CBMAB-EN889-LY
Rating: 5 (1 reviews)

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Datasheet

SDS

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Basic Information

Host Animal

Mouse

Clone

EG735

Application

WB, IHC

Immunogen

Human CLN6.

Host Species

Mouse

Specificity

Human

Antibody Isotype

IgG2a, κ

Clonality

Monoclonal Antibody

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

Buffer

PBS

Preservative

None

Concentration

1 mg/ml

Storage

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

More Infomation

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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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

Isotype control

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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