CLN6 Polyclonal Antibody

Product Specifications

Background

CLN6, a 311 amino acid protein, has seven 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.

Synonyms

Ceroid lipofuscinosis, neuronal 6, late infantile, variant; CLN6 protein; FLJ20561; Nclf; Protein CLN6; CLN6_HUMAN.

Gene ID

54982

Swiss Prot

Q9NWW5

Cellular Locus

Cytoplasm, Cell membrane

Host

Rabbit

Immunogen

101-200/311

Target

CLN6

Clonality

Polyclonal

Isotype

IgG

Conjugation

Unconjugated

Source

KLH conjugated synthetic peptide derived from human CLN6

Applications

WB, ELISA, IHC-P, IHC-F, IF (IHC-P), IF (IHC-F), IF (ICC)

Purification

Purified by Protein A.

Concentration

1µg/µl

Dilution

WB (1:300-5000), ELISA (1:500-1000), IHC-P (1:200-400), IHC-F (1:100-500), IF (IHC-P) (1:50-200), IF (IHC-F) (1:50-200), IF (ICC) (1:50-200)

Buffer

0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Modification

Unmodified

Storage Conditions

Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.

Gene ID URL

54982

Predicted Cross Reactivity

Human, Mouse, Rat, Cow, Sheep, Pig, Horse, Rabbit

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