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CLN8 Antibody

CAT: 0223-26-399Size: 100 µLDry Ice: NoHazardous: No
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CAT#:0223-26-399Size:100 µL
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Background
CLN8 is a transmembrane protein belonging to a family of proteins containing TLC domains, which are postulated to function in lipid synthesis, transport, or sensing. The protein localizes to the endoplasmic reticulum (ER), and may recycle between the ER and ER-Golgi intermediate compartment. Mutations in this gene are associated with progressive epilepsy with mental retardation (EMPR), which is a subtype of neuronal ceroid lipofuscinoses (NCL) . Patients with mutations in this gene have altered levels of sphingolipid and phospholipids in the brain. Childhood-onset NCL are a group of autosomal recessive progressive encephalopathies characterized by the accumulation of autofluorescent material, mainly ATP synthase subunit C, in various tissues, notably in neurons. Based on clinical features, the country of origin of patients, and the molecular genetic background of the disorder, at least seven different forms are thought to exist. CLN8 is characterized by normal early development, onset of generalized seizures between 5 and 10 years, and subsequent progressive mental retardation.This gene encodes a transmembrane protein belonging to a family of proteins containing TLC domains, which are postulated to function in lipid synthesis, transport, or sensing. The protein localizes to the endoplasmic reticulum (ER), and may recycle between the ER and ER-Golgi intermediate compartment. Mutations in this gene are associated with progressive epilepsy with mental retardation (EMPR), which is a subtype of neuronal ceroid lipofuscinoses (NCL) . Patients with mutations in this gene have altered levels of sphingolipid and phospholipids in the brain.
NCBI Gene ID
2055
Swiss Prot
Q9UBY8
Accession Number
NP_061764
Host
Rabbit
Reactivity
Human, Dog
Immunogen
Antibody produced in rabbits immunized with a synthetic peptide corresponding a region of human CLN8.
Clonality
Polyclonal
Conjugation
Unconjugated
Type
Primary Antibodies
Field of Research
Membrane, Neuroscience
Purification
Antibody is purified by peptide affinity chromatography method.
Positive Control
Cat. No. 1201 - HeLa Cell Lysate
Concentration
Batch dependent
Buffer
Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
Modification
None
Shipping Conditions
Blue Ice
Storage Conditions
For short periods of storage (days) store at 4˚ C. For longer periods of storage, store CLN8 antibody at -20˚ C. As with any antibody avoid repeat freeze-thaw cycles.
Calculated Molecular Weight
33 kDa
Applications Notes
CLN8 antibody can be used for detection of CLN8 by ELISA at 1:12500. CLN8 antibody can be used for detection of CLN8 by western blot at 1 μ g/mL, and HRP conjugated secondary antibody should be diluted 1:50,000 - 100,000.
Symbol
CLN8
NCBI Official Name
Ceroid-lipofuscinosis, neuronal 8 (epilepsy, progressive with mental retardation)
NCBI Organism
Homo sapiens
Background Reference 01
Hermansson, M., (2005) J. Neurochem. 95 (3), 609-617.
Other Product Names
CLN8, C8orf61, EPMR, FLJ39417
Tested Applications
ELISA, WB
Protein ID
31083053
Physical Properties
Liquid

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