CLN8 antibody (N-Term)

Catalog No. ABIN406046

The Rabbit Polyclonal anti-CLN8 antibody is suitable to detect CLN8 in samples from Human, Dog, Guinea Pig and Horse. It has been validated for WB.

Quick Overview for CLN8 antibody (N-Term) (ABIN406046)

Target: CLN8 (Ceroid-Lipofuscinosis, Neuronal 8 (Epilepsy, Progressive with Mental Retardation) (CLN8))

Reactivity: Human, Dog, Guinea Pig, Horse

Host: Rabbit

Clonality: Polyclonal

Conjugate: This CLN8 antibody is un-conjugated

Application: Western Blotting (WB)

Product Details anti-CLN8 Antibody

Binding Specificity: N-Term

Purpose: CLN8 Antibody - N-terminal region

Sequence: MNPASDGGTS ESIFDLDYAS WGIRSTLMVA GFVFYLGVFV VCHQLSSSLN

Predicted Reactivity: Dog: 100%, Guinea Pig: 86%, Horse: 100%, Human: 100%

Characteristics: This is a rabbit polyclonal antibody against CLN8. It was validated on Western Blot using a cell lysate as a positive control.

Purification: Affinity Purified

Immunogen: The immunogen is a synthetic peptide directed towards the N terminal region of human CLN8

Application Details

Application Notes: Optimal working dilution should be determined by the investigator.

Comment:

We also sell a specific blocking peptide that can be used in combination with this antibody. You can find the blocking peptide under AAP49408-100UG

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 0.5 mg/mL

Buffer: Purified antibody supplied in 1x PBS buffer with 0.09 % (w/v) sodium azide and 2 % sucrose.

Preservative: Sodium azide

Precaution of Use: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Handling Advice: prevent freeze-thaw cycles

Storage: 4 °C,-20 °C

Storage Comment: For short term use, store at 2-8C up to 1 week. For long term storage, store at -20 °C in small aliquots to prevent freeze-thaw cycles.

Target Details for CLN8

Target: CLN8 (Ceroid-Lipofuscinosis, Neuronal 8 (Epilepsy, Progressive with Mental Retardation) (CLN8))

Alternative Name: CLN8

Background:

Background Information: 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.

Gene Name: Ceroid-lipofuscinosis, neuronal 8 (epilepsy, progressive with mental retardation)

Alternative Symbols: EPMR, TLCD6, C8orf61

Protein Name: Protein CLN8

Molecular Weight: 33kDa

Gene ID: 2055

NCBI Accession: NP_061764

UniProt: Q9UBY8

Pathways: Regulation of Cell Size, Dicarboxylic Acid Transport