Kufs disease Type B is caused by mutations to the DNAJC5 and CTSF genes.[3] This form of Kufs is autosomal dominant, meaning that only one copy of each mutated gene is enough for the disease to manifest. When there is a mutation in the DNAJC5 gene, it affects the production of a cysteine string protein (CSP) that is coded within DNAJC5.[4] CSP aids in transmitting signals through the nerves found in the brain. When the CTSF gene is mutated, it cannot produce Cathepsin F - an enzyme that cuts proteins in the lysozyme.[5] By cutting proteins, Cathepsin F can modify the function of the proteins as well as help break them down.