CLN8 Polyclonal Antibody

Product Specifications

Background

CLN8, a 286 amino acid transmembrane protein, localizes mainly to the endoplasmic reticulum, but also partially to the ER-Golgi intermediate compartment (ERGIC) . Mutations in the CLN8 gene cause neuronal ceroid lipofuscinosis 8 and progressive epilepsy with mental retardation (EPMR) . Both disorders are forms of neuronal ceroid-lipofuscinose (NCL), a group of progressive neurodegenerative diseases found in children, characterized by failure of psychomotor development, impaired vision, seizures and premature death. The CLN8 protein is one of eight proteins in the CLN family, including CLN1-CLN7, which are associated with NCL.

Synonyms

Ceroid-lipofuscinosis, neuronal 8 epilepsy, progressive with mental retardation; Cln8; CLN8_HUMAN; EPMR; Protein CLN8; C8orf61

Gene ID

2055

Swiss Prot

Q9UBY8

Cellular Locus

Cytoplasm, Cell membrane

Host

Rabbit

Cross Reactivity

Human, Mouse, Rat

Immunogen

201-286/286

Target

CLN8

Clonality

Polyclonal

Conjugation

Unconjugated

Source

KLH conjugated synthetic peptide derived from human CLN8

Applications

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

Purification

Purified by Protein A.

Concentration

1ug/ul

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

Prediction Reactivity

Dog, Pig, Horse, Rabbit

Gene ID URL

2055

Isotype

IgG

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