CLN8 rabbit pAb

Ceroid-lipofuscinosis, neuronal 8 (CLN8) Homo sapiens 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. [provided by RefSeq, Jul 2008],

Product Specifications

Background

Ceroid-lipofuscinosis, neuronal 8 (CLN8) Homo sapiens 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. [provided by RefSeq, Jul 2008]

UniProt

Q9UBY8

Swiss Prot

Q9UBY8

Reactivity

Human; Rat; Mouse

Immunogen

Synthesized peptide derived from human protein . at AA range: 231-280

Target

CLN8

Clonality

Polyclonal

Source

Rabbit

Applications

WB; ELISA

Concentration

1 mg/ml

Dilution

WB 1:500-2000 ELISA 1:5000-20000

Buffer

-20°C/1 year

Molecular Weight

31kD

Storage Conditions

-20°C/1 year

Observed Molecular Weight

31kD

Fragment

IgG

Subcellular Location

Endoplasmic reticulum membrane ; Multi-pass membrane protein . Endoplasmic reticulum-Golgi intermediate compartment membrane ; Multi-pass membrane protein . Endoplasmic reticulum .