CLN8 Antibody
| Code | Size | Price |
|---|
| PSI-26-399-100ul | 100ul | £662.00 |
Quantity:
Prices exclude any Taxes / VAT
Overview
Host Type: Rabbit
Antibody Clonality: Polyclonal
Regulatory Status: RUO
Target Species:
- Canine (dog)
- Human
Applications:
- Enzyme-Linked Immunosorbent Assay (ELISA)
- Western Blot (WB)
Storage:
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.
Documents
Further Information
Additional Names:
CLN8, C8orf61, EPMR, FLJ39417
Application Note:
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 μg/mL, and HRP conjugated secondary antibody should be diluted 1:50,000 - 100,000.
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.
Background References:
- Hermansson, M., (2005) J. Neurochem. 95 (3), 609-617.
Buffer:
Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
Concentration:
batch dependent
Conjugate:
Unconjugated
Immunogen:
Antibody produced in rabbits immunized with a synthetic peptide corresponding a region of human CLN8.
NCBI Gene ID #:
2055
NCBI Official Name:
ceroid-lipofuscinosis, neuronal 8 (epilepsy, progressive with mental retardation)
NCBI Official Symbol:
CLN8
NCBI Organism:
Homo sapiens
Physical State:
Liquid
PREDICTED MOLECULAR WEIGHT:
33 kDa
Protein Accession #:
NP_061764
Protein GI Number:
31083053
Purification:
Antibody is purified by peptide affinity chromatography method.
Research Area:
Membrane ,Neuroscience
Swissprot #:
Q9UBY8
User NOte:
Optimal dilutions for each application to be determined by the researcher.