CLN6 antibody

Catalog No. ABIN317825

Product Details anti-CLN6 Antibody

Target: CLN6 (Ceroid-Lipofuscinosis, Neuronal 6, Late Infantile, Variant (CLN6))

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This CLN6 antibody is un-conjugated

Application: Western Blotting (WB), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))

Specificity: CLN6 antibody detects endogenous levels of CLN6 protein.(region surrounding asn258)

Purification: Affinity chromatography

Application Details

Application Notes: ELISA: 1: 40000approx. 1: 60000. WB: 1: 500approx. 1: 1000. IHC: 1: 50approx. 1: 200.
Other applications not tested.
Optimal dilutions are dependent on conditions and should be determined by the user.

Restrictions: For Research Use only

Handling

Concentration: 1,0 mg/mL

Buffer: Phosphate buffered saline (PBS) with 0.05 % sodium azide, approx. pH 7.2.

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: Avoid repeated freezing and thawing.

Storage: 4 °C/-20 °C

Storage Comment: Store the antibody undiluted at 2-8 °C for one month or (in aliquots) at -20 °C for longer.

Target Details for CLN6

Target: CLN6 (Ceroid-Lipofuscinosis, Neuronal 6, Late Infantile, Variant (CLN6))

Alternative Name: CLN6 (CLN6 Products)

Synonyms: 1810065L06Rik antibody, AW743417 antibody, D9Bwg1455e antibody, nclf antibody, CLN4A antibody, HsT18960 antibody, cln6 antibody, zgc:103565 antibody, ceroid-lipofuscinosis, neuronal 6 antibody, CLN6, transmembrane ER protein antibody, CLN6, transmembrane ER protein S homeolog antibody, ceroid-lipofuscinosis, neuronal 6, late infantile, variant antibody, CLN6, transmembrane ER protein a antibody, Cln6 antibody, CLN6 antibody, cln6.S antibody, cln6a antibody

Background: CLN6, a 311-amino acid protein, has 7 predicted transmembrane domains and is conserved across vertebrates. The CLN6 protein localizes to the endoplasmic reticulum but contributes to lysosomal function. Mutations in the CLN6 gene cause variant late-onset infantile neuronal ceroid lipofuscinosis (vLINCL), a lysosomal storage disorder marked by progressive mental deterioration and blindness, part of a group of severe inherited neurodegenerative disorders affecting children wherein lysosomes accumulate storage material, causing the death of neurons. CLN6 is one of eight proteins, including CLN1-8, that are associated with NCL.Synonyms: Ceroid-lipofuscinosis neuronal protein 6

Molecular Weight: approx. 40 kDa

Gene ID: 54982

NCBI Accession: NP_060352

UniProt: Q9NWW5

Pathways: Glycosaminoglycan Metabolic Process