is caused by a mutation in the MITF gene. The mutation in MITF deletes or changes a single amino acid base pair specifically in the base motif region of the MITF protein. The new MITF protein is unable to bind to DNA and melanocyte development and subsequently melanin production is altered. A reduced number of melanocytes can lead to hearing loss, and decreased melanin production can account for the light skin and hair color that make Tietz syndrome so noticeable. Melanoma Melanocytes are commonly known as cells that are responsible for producing the pigment melanin which gives coloration to the hair, skin, and nails. The exact mechanisms of how exactly melanocytes become cancerous are relatively unclear, but there is ongoing research to gain more information about the process. For example, it has been uncovered that the DNA of certain genes is often damaged in melanoma cells, most likely as a result of damage from UV radiation, and in turn increases the likelihood of developing melanoma. Specifically, it has been found that a large percentage of melanomas have mutations in the B-RAF gene which leads to melanoma by causing an MEK-ERK kinase cascade when activated. In addition to B-RAF, MITF is also known to play a crucial role in melanoma progression. Since it is a transcription factor that is involved in the regulation of genes related to invasiveness, migration, and metastasis, it can play a role in the progression of melanoma. Figure 1 shows the specific activators and targets of MITF that are related to the survival, migration, proliferation, invasion and metastasis of melanoma cells. Target genes MITF recognizes E-box (CAYRTG) and M-box (TCAYRTG or CAYRTGA) sequences in the promoter regions of target genes. Known target genes (confirmed by at least two independent sources) of this transcription factor include, Additional genes identified by a microarray study (which confirmed the above targets) include the following, The LysRS-Ap4A-MITF signaling pathway The LysRS-Ap4A-MITF signaling pathway was first discovered in mast cells, in which, the A mitogen-activated protein kinase (MAPK) pathway is activated upon allergen stimulation. The binding of immunoglobulin E to the high-affinity IgE receptor (FcεRI) provides the stimulus that starts the cascade. Lysyl-tRNA synthetase (LysRS) normally resides in the multisynthetase complex. This complex consists of nine different aminoacyl-tRNA synthetases and three scaffold proteins and has been termed the "signalosome" due to its non-catalytic signalling functions. After activation, LysRS is phosphorylated on Serine 207