CLN6 Antilichaam - middengebied: FITC (ARP47371 P050-FITC)

Ceroid-lipofuscinosis, neuronal 6, late infantile, variant

Nclf, CLN4A, HsT18960

54982

Q9NWW5

NP_060352

Human, Mouse, Rat, Cow, Dog, Guinea Pig, Horse, Rabbit, Sheep, Zebrafish

The immunogen is a synthetic peptide directed towards the middle region of human CLN6

CLN6 is one of eight which have been associated with neuronal ceroid lipofuscinoses (NCL) . Also referred to as Batten disease, NCL comprises a class of autosomal recessive, neurodegenerative disorders affecting children. The genes responsible likely CLN6 involved in the degradation of post-translationally modified proteins in lysosomes. The primary defect in NCL disorders is thought to be associated with lysosomal storage function.

RNF2; BMI1; ILK; env; DERL1; VCP; UBC

FITC: Fluorescein Isothiocyanate

Polyclonal Antibody

WB

Affinity Purified

0.5 mg/ml

Cow: 93%; Dog: 100%; Guinea Pig: 100%; Horse: 100%; Human: 100%; Mouse: 100%; Rabbit: 93%; Rat: 100%; Sheep: 93%; Zebrafish: 93%

Liquid. Purified antibody supplied in 1x PBS buffer.

All conjugated antibodies should be stored in light-protected vials or covered with a light protecting material (i.e. aluminum foil) . Conjugated antibodies are stable for at least 12 months at 4C. If longer storage is desired (24 months), conjugates may be diluted with up to 50% glycerol and stored at -20C to -80C. Freezing and thawing conjugated antibodies will compromise enzyme activity as well as antibody binding.

36kDa

Heine, C., (2007) Mol. Membr. Biol. 24 (1), 74-87

Wet Ice

311

CLN6

CLN6

Rabbit

Ceroid-lipofuscinosis neuronal protein 6

NM_017882

LPRSITYVSIIIFIMGASIHLVGDSVNHRLLFSGYQHHLSVRENPIIKNL