PPT1 is a small glycoprotein involved in the catabolism of lipid-modified proteins during lysosomal degradation.
The encoded enzyme removes thioester-linked 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.
The cDNA predicted a 306aa polypeptide that contains a 25aa signal peptide, 3 N-linked glycosylation sites, and consensus motifs characteristic of thioesterases.
Northern blot analysis revealed ubiquitous expression of a single 2.5-kb mRNA, with highest expression in lung, brain, and heart.
Applications:Suitable for use in ELISA, Western Blot, Immunohistochemistry, Immunocytochemistry.
Other applications not tested.
Recommended Dilution:ELISA: 1:100-1:5000 Western Blot: 1:50-400 Immunohistochemistry (frozen): 1:50-500 Immunohistochemistry (paraffin): 1:10-100 Immunocytochemistry: 1:50-500 Optimal dilutions to be determined by the researcher.