Reactive species
Human,Mouse,Rat
Immunogen
Recombinant fusion protein containing a sequence corresponding to amino acids 28-306 of
human PPT1 (NP_000301.1).
Dilution rate
WB 1:500 - 1:2000
Purification process
Affinity purification
Stockpile
Store at -20℃. Avoid freeze / thaw cycles.
Buffer: PBS with 0.02% sodium azide,50% glycerol,pH7.3.
Other name
PPT; CLN1; INCL; PPT1
Background
The protein encoded by this gene is a small glycoprotein involved in the catabolism of lipidmodified proteins during lysosomal degradation. The encoded enzyme removes thioesterlinked fatty acyl groups such as palmitate from cysteine residues. Defects in this gene are a
cause of infantile neuronal ceroid lipofuscinosis 1 (CLN1, or INCL) and neuronal ceroid
lipofuscinosis 4 (CLN4). Two transcript variants encoding different isoforms have been found
for this gene.
