CLN8 antibody - N-terminal region
Produktgrößen
25ul
£177,00
ARP49408-P050-25UL
100 ul
£412,00
ARP49408-P050-100UL
Über dieses Produkt
- SKU:
- ARP49408-P050
- Zusätzliche Namen:
- EPMR; TLCD6; C8orf61
- Klonalität:
- Polyclonal
- Konzentration:
- 0.5 mg/ml
- Weitere Details:
- 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.
- Gendetails:
- Ceroid-lipofuscinosis; neuronal 8 (epilepsy; progressive with mental retardation)
- Host:
- Rabbit
- Immunogen:
- The immunogen is a synthetic peptide directed towards the N terminal region of human CLN8
- Molekulargewicht:
- 33kDa
- Proteindetails:
- Protein CLN8
- Aufreinigung:
- Affinity Purified
- Versandbedingungen:
- Blue Ice
- Lagerbedingungen:
- For short term use; store at 2-8C up to 1 week. For long term storage; store at -20C in small aliquots to prevent freeze-thaw cycles.
- Hersteller:
- Aviva Systems Biology
- Typ:
- Antibodies: Polyclonal Antibody
- Datenblatt des Herstellers:html_datasheet.php