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CLN5, Polyclonal Antibody

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产品名称: CLN5, Polyclonal Antibody
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简单介绍

CLN5, Polyclonal Antibody


CLN5, Polyclonal Antibody  的详细介绍
Product Name

CLN5, Polyclonal Antibody

Full Product Name

Anti-CLN5 Antibody

Product Synonym Names
Ceroid-lipofuscinosis neuronal protein 5; Protein CLN5
Product Gene Name

anti-CLN5 antibody

[Similar Products]
Antibody/Peptide Pairs
CLN5 peptide (MBS8210779) is used for blocking the activity of CLN5 antibody (MBS8209559)
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
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OMIM
256731
3D Structure
ModBase 3D Structure for O75503
Clonality
Polyclonal
Host
Rabbit
Species Reactivity
Human, Mouse, Bovine, Sheep
Specificity
Recognizes endogenous levels of CLN5 protein.
Purity/Purification
The antibody was purified by immunogen affinity chromatography.
Form/Format
Liquid in 0.42% Potassium phosphate, 0.87% Sodium chloride, pH 7.3, 30% glycerol, and 0.01% sodium azide.
Immunogen
KLH-conjugated synthetic peptide encompassing a sequence within the center region of human CLN5. The exact sequence is proprietary.
Preparation and Storage
Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C for one year. Avoid freeze/thaw cycles.
Other Notes
Small volumes of anti-CLN5 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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Related Product Information for
anti-CLN5 antibody
Rabbit polyclonal antibody to CLN5
Applications Tested/Suitable for anti-CLN5 antibody
Western Blot (WB)
Application Notes for anti-CLN5 antibody
WB (1/500 - 1/1000)

Western Blot (WB) of anti-CLN5 antibody
Western blot analysis of CLN5 expression in HEK293T (A), NIH3T3 (B) whole cell lysates.
anti-CLN5 antibody Western Blot (WB) (WB) image
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NCBI/Uniprot data below describe general gene information for CLN5. It may not necessarily be applicable to this product.
NCBI GI #
5729772
NCBI GeneID
1203
NCBI Accession #
NP_006484.1 [Other Products]
NCBI GenBank Nucleotide #
NM_006493.2 [Other Products]
UniProt Primary Accession #
O75503 [Other Products]
UniProt Secondary Accession #
B3KQK7[Other Products]
UniProt Related Accession #
O75503[Other Products]
Molecular Weight
41,497 Da
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NCBI Official Full Name
ceroid-lipofuscinosis neuronal protein 5
NCBI Official Synonym Full Names
ceroid-lipofuscinosis, neuronal 5
NCBI Official Symbol
CLN5  [Similar Products]
NCBI Official Synonym Symbols
NCL
  [Similar Products]
NCBI Protein Information
ceroid-lipofuscinosis neuronal protein 5
UniProt Protein Name
Ceroid-lipofuscinosis neuronal protein 5
Protein Family
Cln5-like protein
UniProt Gene Name
CLN5  [Similar Products]
UniProt Synonym Gene Names
Protein CLN5  [Similar Products]
UniProt Entry Name
CLN5_HUMAN
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NCBI Summary for CLN5
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]
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UniProt Comments for CLN5
CLN5: Defects in CLN5 are the cause of neuronal ceroid lipofuscinosis type 5 (CLN5); also known as Finnish variant late-infantile neuronal ceroid lipofuscinosis (vLINCL). 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 5 comprise mixed combinations of granular, curvilinear, and fingerprint profiles. Belongs to the CLN5 family.

Protein type: Membrane protein, integral

Chromosomal Location of Human Ortholog: 13q21.1-q32

Cellular Component: Golgi apparatus; lysosomal membrane; endoplasmic reticulum; lysosome; perinuclear region of cytoplasm; integral to membrane

Molecular Function: mannose binding; protein binding

Biological Process: signal peptide processing; neurogenesis; lysosomal lumen acidification; protein catabolic process; brain development; neuron maturation

Disease: Ceroid Lipofuscinosis, Neuronal, 5
Research Articles on CLN5
1. There are functional differences in various N-glycosylation sites of CLN5 which affect folding, trafficking, and lysosomal function of CLN5.
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Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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