Transmembrane Protein 135 (TMEM135)

Disorders of mitochondrial fat metabolism lead to sudden death in infants and children. Although survival is possible, the underlying molecular mechanisms which enable this outcome have not yet been clearly identified. TMEM135, a conserved protein upregulated 4.3 (±0.14)-fold in VLCAD-deficient mice at birth.

TMEM135 is highly expressed in mitochondria and in fat-loaded tissues in the mouse. TMEM135 integrates biological processes involving fat metabolism and energy expenditure in both the worm (invertebrates) and in mammalian organisms. TMEM135 is part of a regulatory circuit that plays a critical role in the survival of VLCAD-deficient mice and perhaps in other mitochondrial genetic defects of fat metabolism as well.