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Proteintech
CLN6 Polyclonal Antibody
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Product Details
20315-1-AP
Applications
Tested Dilution
Publications
Product Specifications
Species Reactivity
Human,
Mouse
Published species
Human
Host/Isotype
Rabbit
/ IgG
Class
Polyclonal
Type
Antibody
Immunogen
CLN6 Fusion Protein Ag14159 (121-190 aa encoded by BC010849)
Conjugate
Form
Liquid
Concentration
0.51 mg/mL
Purification
Antigen affinity chromatography
Storage buffer
PBS, pH 7.3, with 50% glycerol
Contains
0.02% sodium azide
Storage conditions
-20°C
Shipping conditions
Wet ice (domestic); Dry ice (international)
Product Specific Information
Immunogen sequence: IMGASIHLV GDSVNHRLLF SGYQHHLSVR ENPIIKNLKP ETLIDSFELL YYYDEYLGHC MWYIPFFLIL F (121-190 aa encoded by BC010849)
Target Information
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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Bioinformatics
Protein Aliases: ceroid-lipofuscinosis; ceroid-lipofuscinosis neuronal 6 late infantile; Ceroid-lipofuscinosis neuronal protein 6; neuronal ceroid lipofuscinosis; Protein CLN6
Gene Aliases: 1810065L06Rik; AW743417; CLN4A; CLN6; D9Bwg1455e; HsT18960; nclf
UniProt ID: (Human) Q9NWW5
Entrez Gene ID: (Human) 54982, (Mouse) 76524
ganglioside metabolic process
lysosome organization
lysosomal lumen acidification
visual perception
cholesterol metabolic process
glycosaminoglycan metabolic process
locomotion involved in locomotory behavior
cellular macromolecule catabolic process
positive regulation of proteolysis
protein catabolic process
Performance Guarantee
If an Invitrogen™ antibody doesn't perform as described on our website or datasheet,we'll replace the product at no cost to you, or provide you with a credit for a future purchase.*
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