CLN8 Polyclonal Antibody, AbBy Fluor™ 350 Conjugated
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
AbBy Fluor™ 350
Source
KLH conjugated synthetic peptide derived from human CLN8
Applications
WB, IF (IHC-P), IF (IHC-F), IF (ICC)
Purification
Purified by Protein A.
Excitation Emission
346nm/442nm
Concentration
1ug/ul
Dilution
WB (1:300-5000), IF (IHC-P) (1:50-200), IF (IHC-F) (1:50-200), IF (ICC) (1:50-200)
Buffer
Aqueous buffered solution containing 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Modification
Unmodified
Storage Conditions
Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
Prediction Reactivity
Dog, Pig, Horse, Rabbit
Gene ID URL
2055
Isotype
IgG
Curated Selection
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