CLN-3 protein is expressed in the pancreatic somatostatin-secreting delta cells.
Juvenile neuronal ceroid lipofuscinosis (JNCL) is a severe autosomal recessive neurodegenerative disorder resulting from mutations in the CLN3 gene. The gene product is a 438-amino acid hydrophobic peptide of unknown function containing five transmembrane domains. In order to study the tissue distribution of the peptide, polyclonal antibodies were raised in rabbits to three epitopes and were affinity purified before use. All three antibodies were used together for immunocytochemical staining of human pancreas. This staining showed localization in pancreatic islet cells. Double labelling of the tissue indicated that cells staining for the CLN3 protein were also positive for somatostatin.
Boriack
R L
RL
Department of Pathology, Children's Medical Center of Dallas, 1935 Motor Street, Dallas, Texas 75235, USA.
Bennett
M J
MJ
D000818:Animals; D000906:Antibodies; D002648:Child; D000939:Epitopes / Q000276:immunology; D006801:Humans; D007150:Immunohistochemistry; D008562:Membrane Glycoproteins; D018832:Molecular Chaperones; D009472:Neuronal Ceroid-Lipofuscinoses / Q000378:metabolism*; D014176:Protein Biosynthesis; D011506:Proteins / Q000032:analysis / Q000276:immunology; D011817:Rabbits; D013004:Somatostatin / Q000378:metabolism; D019864:Somatostatin-Secreting Cells / Q000737:chemistry / Q000378:metabolism*
D000906:Antibodies; C105199:CLN3 protein, human; D000939:Epitopes; D008562:Membrane Glycoproteins; D018832:Molecular Chaperones; D011506:Proteins; D013004:Somatostatin