Neuronal Ceroid Lipofuscinoses NCL /CLN are neurodegenerative hereditary disorders of children and adolescents caused by mutations in CLN proteins. The functions of most CLN proteins are unknown. The ER membrane protein CLN6 has been identified as a cause of juvenile NCL and its involvement in growth processes in the Dorsal Root Ganglion, and a function in microtubule expression and intracellular transport is discussed. Specific monoclonal anti-CLN6 antibodies were generated for this work. Spleen cells from immunized mice and myeloma cells were fused to hybridomas and 4 antibody-producing clones were isolated. The antibodies detected recombinant CLN6. In immunofluorescence, the colocalization of CLN6 and the ER-specific Protein Disulfide Isomerase could be detected in a breast cancer cell line. In immunohistology, CLN6 was stained at the protein level in secretory cells of the gastric corpus and in human mammary, liver, kidney and skeletal muscle tissues.